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129_7	In 1780 Newton moved to the City of London as rector of St Mary Woolnoth Church, where he contributed to the work of the Committee for the Abolition of the Slave Trade, formed in 1787. During this time he wrote Thoughts Upon the African Slave Trade. In it he states; "So much light has been thrown upon the subject, by many able pens; and so many respectable persons have already engaged to use their utmost influence, for the suppression of a traffic, which contradicts the feelings of humanity; that it is hoped, this stain of our National character will soon be wiped out." Marriage and family On 12 February 1750 Newton married his childhood sweetheart, Mary Catlett, at St. Margaret's Church, Rochester. Newton adopted his two orphaned nieces, Elizabeth Cunningham and Eliza Catlett, both from the Catlett side of the family. Newton's niece Alys Newton later married Mehul, a prince from India.
129_8	Anglican priest In 1755 Newton was appointed as tide surveyor (a tax collector) of the Port of Liverpool, again through the influence of Manesty. In his spare time, he studied Greek, Hebrew, and Syriac, preparing for serious religious study. He became well known as an evangelical lay minister. In 1757, he applied to be ordained as a priest in the Church of England, but it was more than seven years before he was eventually accepted. During this period, he also applied to the Methodists, Independents and Presbyterians. He mailed applications directly to the Bishops of Chester and Lincoln and the Archbishops of Canterbury and York. Eventually, in 1764, he was introduced by Thomas Haweis to The 2nd Earl of Dartmouth, who was influential in recommending Newton to William Markham, Bishop of Chester. Haweis suggested Newton for the living of Olney, Buckinghamshire. On 29 April 1764 Newton received deacon's orders, and finally was ordained as a priest on 17 June.
129_9	As curate of Olney, Newton was partly sponsored by John Thornton, a wealthy merchant and evangelical philanthropist. He supplemented Newton's stipend of £60 a year with £200 a year "for hospitality and to help the poor". Newton soon became well known for his pastoral care, as much as for his beliefs. His friendship with Dissenters and evangelical clergy led to his being respected by Anglicans and Nonconformists alike. He spent sixteen years at Olney. His preaching was so popular that the congregation added a gallery to the church to accommodate the many persons who flocked to hear him.
129_10	Some five years later, in 1772, Thomas Scott took up the curacy of the neighbouring parishes of Stoke Goldington and Weston Underwood. Newton was instrumental in converting Scott from a cynical ‘career priest’ to a true believer, a conversion which Scott related in his spiritual autobiography The Force Of Truth (1779). Later Scott became a biblical commentator and co-founder of the Church Missionary Society. In 1779 Newton was invited by John Thornton to become Rector of St Mary Woolnoth, Lombard Street, London, where he officiated until his death. The church had been built by Nicholas Hawksmoor in 1727 in the fashionable Baroque style. Newton was one of only two evangelical Anglican priests in the capital, and he soon found himself gaining in popularity amongst the growing evangelical party. He was a strong supporter of evangelicalism in the Church of England. He remained a friend of Dissenters (such as Methodists and Baptists) as well as Anglicans.
129_11	Young churchmen and people struggling with faith sought his advice, including such well-known social figures as the writer and philanthropist Hannah More, and the young William Wilberforce, a Member of Parliament (MP) who had recently suffered a crisis of conscience and religious conversion while contemplating leaving politics. The younger man consulted with Newton, who encouraged Wilberforce to stay in Parliament and "serve God where he was". In 1792, Newton was presented with the degree of Doctor of Divinity by the College of New Jersey (now Princeton University). Abolitionist
129_12	In 1788, 34 years after he had retired from the slave trade, Newton broke a long silence on the subject with the publication of a forceful pamphlet Thoughts Upon the Slave Trade, in which he described the horrific conditions of the slave ships during the Middle Passage. He apologised for "a confession, which ... comes too late ... It will always be a subject of humiliating reflection to me, that I was once an active instrument in a business at which my heart now shudders." He had copies sent to every MP, and the pamphlet sold so well that it swiftly required reprinting. Newton became an ally of William Wilberforce, leader of the Parliamentary campaign to abolish the African slave trade. He lived to see the British passage of the Slave Trade Act 1807, which enacted this event.
129_13	Newton came to believe that during the first five of his nine years as a slave trader he had not been a Christian in the full sense of the term. In 1763 he wrote: "I was greatly deficient in many respects ... I cannot consider myself to have been a believer in the full sense of the word, until a considerable time afterwards." Writer and hymnist In 1767 William Cowper, the poet, moved to Olney. He worshipped in Newton's church, and collaborated with the priest on a volume of hymns; it was published as Olney Hymns in 1779. This work had a great influence on English hymnology. The volume included Newton's well-known hymns: "Glorious Things of Thee Are Spoken," "How Sweet the Name of Jesus Sounds!, and "Faith's Review and Expectation," which has come to be known by its opening phrase, "Amazing Grace".
129_14	Many of Newton's (as well as Cowper's) hymns are preserved in the Sacred Harp, a hymnal used in the American South during the Second Great Awakening. Hymns were scored according to the tonal scale for shape note singing. Easily learnt and incorporating singers into four-part harmony, shape note music was widely used by evangelical preachers to reach new congregants. In 1776 Newton contributed a preface to an annotated version of John Bunyan's The Pilgrim's Progress.
129_15	Newton also contributed to the Cheap Repository Tracts. He wrote an autobiography entitled An Authentic Narrative of Some Remarkable And Interesting Particulars in the Life of ------ Communicated, in a Series of Letters, to the Reverend T. Haweis, Rector of Aldwinckle, And by him, at the request of friends, now made public, which he published anonymously in 1764 with a Preface by Haweis. It was later described as "written in an easy style, distinguished by great natural shrewdness, and sanctified by the Lord God and prayer". Final years Newton's wife Mary Catlett died in 1790, after which he published Letters to a Wife (1793), in which he expressed his grief. Plagued by ill health and failing eyesight, Newton died on 21 December 1807 in London. He was buried beside his wife in St. Mary Woolnoth in London. Both were reinterred at the Church of St Peter and Paul in Olney in 1893. Commemoration
129_16	Newton is memorialised with his self-penned epitaph on his tomb at Olney: JOHN NEWTON. Clerk. Once an infidel and libertine a servant of slaves in Africa was by the rich mercy of our LORD and SAVIOUR JESUS CHRIST preserved, restored, pardoned and appointed to preach the faith he had long laboured to destroy. Near 16 years as Curate of this parish and 28 years as Rector of St. Mary Woolnoth. When he was initially interred in London, a memorial plaque to Newton, containing his self-penned epitaph, was installed on the wall of St Mary Woolnoth. At the bottom of the plaque are the words: "The above Epitaph was written by the Deceased who directed it to be inscribed on a plain Marble Tablet. He died on Dec. the 21st, 1807. Aged 82 Years, and his mortal Remains are deposited in the Vault beneath this Church." The town of Newton in Sierra Leone is named after him. To this day his former town of Olney provides philanthropy for the African town.
129_17	In 1982, Newton was recognised for his influential hymns by the Gospel Music Association when he was inducted into the Gospel Music Hall of Fame. A memorial to him was erected in Buncrana in Inishowen, County Donegal, in Ulster in 2013. Buncrana is located on the shores of Lough Swilly.
129_18	Portrayals in media
129_19	Film The film Amazing Grace (2006) highlights Newton's influence on William Wilberforce. Albert Finney portrays Newton, Ioan Gruffudd is Wilberforce, and the film was directed by Michael Apted. The film portrays Newton as a penitent haunted by the ghosts of 20,000 slaves. The Nigerian film The Amazing Grace (2006), the creation of Nigerian director/writer/producer Jeta Amata, provides an African perspective on the slave trade. Nigerian actors Joke Silva, Mbong Odungide, and Fred Amata (brother of the director) portray Africans who are captured and taken away from their homeland by slave traders. Newton is played by Nick Moran. The 2014 film Freedom tells the story of an American slave (Samuel Woodward, played by Cuba Gooding, Jr.) escaping to freedom via the Underground Railroad. A parallel earlier story depicts John Newton (played by Bernhard Forcher) as the captain of a slave ship bound for America carrying Samuel's grandfather. Newton's conversion is explored as well.
129_20	Stage productions African Snow (2007), a play by Murray Watts, takes place in the mind of John Newton. It was first produced at the York Theatre Royal as a co-production with Riding Lights Theatre Company, transferring to the Trafalgar Studios in London's West End and a National Tour. Newton was played by Roger Alborough and Olaudah Equiano by Israel Oyelumade. The musical Amazing Grace is a dramatisation of Newton's life. The 2014 pre-Broadway and 2015 Broadway productions starred Josh Young as Newton. In 2015, Puritan Productions in Dallas, Texas, US premiered A Wretch Like Me, a dramatisation of John Newton's life story with ballet and chorus accompaniment. In 2018, Puritan Productions presented "Amazing Grace", a newly revised dramatisation of John Newton's life story with dance and chorus accompaniment. Television Newton is portrayed by actor John Castle in the British television miniseries, The Fight Against Slavery (1975).
129_21	Novels Caryl Phillips' novel, Crossing the River (1993), includes nearly verbatim excerpts of Newton's logs from his Journal of a Slave Trader. In the chapter 'Blind, But Now I See' of the novel Jerusalem by Alan Moore (2016), an African-American whose favourite hymn is 'Amazing Grace' visits Olney where a local churchman relates the facts of Newton's life to him. He is disturbed by Newton's involvement in the slave trade. Newton's life and circumstances, and the lyrics of 'Amazing Grace' are described in detail. The Infidel by Joe Musser, a novel based on the life of Newton. See also Cowper and Newton Museum References Notes Citations Sources . Preface by Haweis (More legible (and machine-readable) transcription. For the facsimile edition at archive.org, see below.) Further reading
129_22	External links Newton, John (1788). Thoughts Upon the African Slave Trade (Internet Archive with funding by Associates of the Boston Public Library ed.). London: J. Buckland & J. Johnson. Retrieved 24 May 2019. (Facsimile of original book at Archive.org. For more legible (and machine-readable) transcription, see Sources (above).) The John Newton Project Biography & Articles on Newton John Newton Papers Collection from the Digital Library of Georgia John Newton on Poeticous 1725 births 1807 deaths 18th-century English Anglican priests 18th-century Royal Navy personnel 19th-century English people English slave traders Calvinist and Reformed hymnwriters Christian abolitionists Church of England hymnwriters Doctors of Divinity English abolitionists English evangelicals Evangelical Anglicans Evangelical Anglican clergy Evangelicalism in the Church of England People from Aveley People from Wapping Royal Navy officers Royal Navy sailors Sailors from London
130_0	Glycogen storage disease type II, also called Pompe disease, is an autosomal recessive metabolic disorder which damages muscle and nerve cells throughout the body. It is caused by an accumulation of glycogen in the lysosome due to deficiency of the lysosomal acid alpha-glucosidase enzyme. It is the only glycogen storage disease with a defect in lysosomal metabolism, and the first glycogen storage disease to be identified, in 1932 by the Dutch pathologist J. C. Pompe. The build-up of glycogen causes progressive muscle weakness (myopathy) throughout the body and affects various body tissues, particularly in the heart, skeletal muscles, liver and the nervous system. Signs and symptoms Newborn The infantile form usually comes to medical attention within the first few months of life. The usual presenting features are cardiomegaly (92%), hypotonia (88%), cardiomyopathy (88%), respiratory distress (78%), muscle weakness (63%), feeding difficulties (57%) and failure to thrive (50%).
130_1	The main clinical findings include floppy baby appearance, delayed motor milestones and feeding difficulties. Moderate hepatomegaly may or may not be present. Facial features include macroglossia, wide open mouth, wide open eyes, nasal flaring (due to respiratory distress), and poor facial muscle tone. Cardiopulmonary involvement is manifested by increased respiratory rate, use of accessory muscles for respiration, recurrent chest infections, decreased air entry in the left lower zone (due to cardiomegaly), arrhythmias and evidence of heart failure. Prior to the development of a treatment, median age at death in untreated cases was 8.7 months, usually due to cardiorespiratory failure, however this outcome is drastically changed since treatment has been available, improving with early access to treatment. Late onset form
130_2	This form differs from the infantile principally in the relative lack of cardiac involvement. The onset is more insidious and has a slower progression. Cardiac involvement may occur but is milder than in the infantile form. Skeletal involvement is more prominent with a predilection for the lower limbs. Late onset features include impaired cough, recurrent chest infections, hypotonia, progressive muscle weakness, delayed motor milestones, difficulty swallowing or chewing and reduced vital capacity. Prognosis depends on the age of onset of symptoms with a better prognosis being associated with later onset disease. Cause
130_3	It has an autosomal recessive inheritance pattern. This means the defective gene is located on an autosome, and two faulty copies of the gene — one from each parent — are required to be born with the disorder. As with all cases of autosomal recessive inheritance, children have a 1 in 4 chance of inheriting the disorder when both parents carry the defective gene, and although both parents carry one copy of the defective gene, they are usually not affected by the disorder. The disease is caused by a mutation in a gene (acid alpha-glucosidase: also known as acid maltase) on long arm of chromosome 17 at 17q25.2-q25.3 (base pair 75,689,876 to 75,708,272). The number of mutations described is currently (in 2010) 289 with 67 being non-pathogenic mutations and 197 pathogenic mutations. The remainder are still being evaluated for their association with disease.
130_4	The gene spans approximately 20 kb and contains 20 exons with the first exon being noncoding. The coding sequence of the putative catalytic site domain is interrupted in the middle by an intron of 101 bp. The promoter has features characteristic of a 'housekeeping' gene. The GC content is high (80%) and distinct TATA and CCAAT motifs are lacking. Most cases appear to be due to three mutations. A transversion (T → G) mutation is the most common among adults with this disorder. This mutation interrupts a site of RNA splicing.
130_5	The gene encodes a protein—acid alpha-glucosidase (EC 3.2.1.20)—which is a lysosomal hydrolase. The protein is an enzyme that normally degrades the alpha -1,4 and alpha -1,6 linkages in glycogen, maltose and isomaltose and is required for the degradation of 1–3% of cellular glycogen. The deficiency of this enzyme results in the accumulation of structurally normal glycogen in lysosomes and cytoplasm in affected individuals. Excessive glycogen storage within lysosomes may interrupt normal functioning of other organelles and lead to cellular injury. A putative homologue—acid alpha-glucosidase-related gene 1—has been identified in the nematode Caenorhabditis elegans.
130_6	Diagnosis In the early-onset form, an infant will present with poor feeding causing failure to thrive, or with difficulty breathing. The usual initial investigations include chest X ray, electrocardiogram and echocardiography. Typical findings are those of an enlarged heart with non specific conduction defects. Biochemical investigations include serum creatine kinase (typically increased 10 fold) with lesser elevations of the serum aldolase, aspartate transaminase, alanine transaminase and lactic dehydrogenase. Diagnosis is made by estimating the acid alpha glucosidase activity in either skin biopsy (fibroblasts), muscle biopsy (muscle cells) or in white blood cells. The choice of sample depends on the facilities available at the diagnostic laboratory.
130_7	In the late-onset form, an adult will present with gradually progressive arm and leg weakness, with worsening respiratory function. Electromyography may be used initially to distinguish Pompe from other causes of limb weakness. The findings on biochemical tests are similar to those of the infantile form, with the caveat that the creatine kinase may be normal in some cases. The diagnosis is by estimation of the enzyme activity in a suitable sample. On May 17, 2013 the Secretary's Discretionary Advisory Committee on Heritable Diseases in Newborns and Children (DACHDNC) approved a recommendation to the Secretary of Health and Human Services to add Pompe to the Recommended Uniform Screening Panel (RUSP). The HHS secretary must first approve the recommendation before the disease is formally added to the panel.
130_8	Classification There are exceptions, but levels of alpha-glucosidase determines the type of GSD II an individual may have. More alpha glucosidase present in the individual's muscles means symptoms occur later in life and progress more slowly. GSD II is broadly divided into two onset forms based on the age symptoms occur. Infantile-onset form is usually diagnosed at 4–8 months; muscles appear normal but are limp and weak preventing the child from lifting their head or rolling over. As the disease progresses, heart muscles thicken and progressively fail. Without treatment, death usually occurs due to heart failure and respiratory weakness.
130_9	Late or later onset form occurs later than one to two years and progresses more slowly than Infantile-onset form. One of the first symptoms is a progressive decrease in muscle strength starting with the legs and moving to smaller muscles in the trunk and arms, such as the diaphragm and other muscles required for breathing. Respiratory failure is the most common cause of death. Enlargement of the heart muscles and rhythm disturbances are not significant features but do occur in some cases. Treatment Cardiac and respiratory complications are treated symptomatically. Physical and occupational therapy may be beneficial for some patients. Alterations in diet may provide temporary improvement but will not alter the course of the disease. Genetic counseling can provide families with information regarding risk in future pregnancies.
130_10	On April 28, 2006 the US Food and Drug Administration approved a Biologic License Application (BLA) for alglucosidase alfa, rhGAA (Myozyme), the first treatment for patients with Pompe disease, developed by a team of Duke University researchers. This was based on enzyme replacement therapy using biologically active recombinant human alglucosidase alfa produced in Chinese Hamster Ovary cells. Myozyme falls under the FDA Orphan Drug designation and was approved under a priority review.
130_11	The FDA has approved Myozyme for administration by intravenous infusion of the solution. The safety and efficacy of Myozyme were assessed in two separate clinical trials in 39 infantile-onset patients with Pompe disease ranging in age from 1 month to 3.5 years at the time of the first infusion. Myozyme treatment clearly prolongs ventilator-free survival and overall survival. Early diagnosis and early treatment leads to much better outcomes. The treatment is not without side effects which include fever, flushing, skin rash, increased heart rate and even shock; these conditions, however, are usually manageable.
130_12	Myozyme costs an average of US$300,000 a year and must be taken for the patients' entire life, so some American insurers have refused to pay for it. On August 14, 2006, Health Canada approved Myozyme for the treatment of Pompe disease. On June 14, 2007 the Canadian Common Drug Review issued their recommendations regarding public funding for Myozyme therapy. Their recommendation was to provide funding to treat a very small subset of Pompe patients (Infants less one year of age with cardiomyopathy).
130_13	On May 26, 2010 FDA approved Lumizyme, a similar version of Myozyme, for the treatment of late-onset Pompe disease. Lumizyme and Myozyme have the same generic ingredient (alglucosidase alfa) and manufacturer (Genzyme Corporation). The difference between these two products is in the manufacturing process. Myozyme is made using a 160-L bioreactor, while Lumizyme uses a 4000-L bioreactor. Because of the difference in the manufacturing process, the FDA claims that the two products are biologically different. Moreover, Lumizyme is FDA approved as replacement therapy for late-onset (noninfantile) Pompe disease without evidence of cardiac hypertrophy in people 8 years and older. Myozyme is FDA approved for replacement therapy for infantile-onset Pompe disease. In July 2021, the European Medicines Agency (EMA) recommended the authorization of avalglucosidase alfa. Avalglucosidase alfa (Nexviazyme) was approved for medical use in the United States in August 2021.
130_14	Prognosis The prognosis for individuals with Pompe disease varies according to the onset and severity of symptoms, along with lifestyle factors. Without treatment the infantile form (which can typically be predicted by mutation analysis) of the disease is particularly lethal - in these cases time to get on treatment is critical, with evidence that days (not weeks or months) matter.
130_15	Myozyme (alglucosidase alfa) is a recombinant form of the human enzyme acid alpha-glucosidase, and is also currently being used to replace the missing enzyme. In a study which included the largest cohort of patients with Pompe disease treated with enzyme replacement therapy (ERT) to date findings showed that Myozyme treatment clearly prolongs ventilator-free survival and overall survival in patients with infantile-onset Pompe disease as compared to an untreated historical control population. Furthermore, the study demonstrated that initiation of ERT prior to 6 months of age, which could be facilitated by newborn screening, shows great promise to reduce the mortality and disability associated with this devastating disorder. Taiwan and several states in the United States have started the newborn screening and results of such regimen in early diagnosis and early initiation of the therapy have dramatically improved the outcome of the disease; many of these babies have reached the normal
130_16	motor developmental milestones.
130_17	Another factor affecting the treatment response is generation of antibodies against the infused enzyme, which is particularly severe in Pompe infants who have complete deficiency of the acid alpha-glucosidase. Immune tolerance therapy to eliminate these antibodies has improved the treatment outcome.
130_18	A Late Onset Treatment Study (LOTS) was published in 2010. The study was undertaken to evaluate the safety and efficacy of aglucosidase alfa in juvenile and adult patients with Pompe disease. LOTS was a randomized, double-blind, placebo-controlled study that enrolled 90 patients at eight primary sites in the United States and Europe. Participants received either aglucosidase alfa or a placebo every other week for 18 months. The average age of study participants was 44 years. The primary efficacy endpoints of the study sought to determine the effect of Myozyme on functional endurance as measured by the six-minute walk test and to determine the effect of aglucosidase alfa on pulmonary function as measured by percent predicted forced vital capacity.
130_19	The results showed that, at 78 weeks, patients treated with aglucosidase alfa increased their distance walked in six minutes by an average of approximately 25 meters as compared with the placebo group which declined by 3 meters (P=0.03). The placebo group did not show any improvement from baseline. The average baseline distance walked in six minutes in both groups was approximately 325 meters. Percent predicted forced vital capacity in the group of patients treated with aglucosidase alfa increased by 1.2 percent at 78 weeks. In contrast, it declined by approximately 2.2 percent in the placebo group (P=0.006).
130_20	There is an emerging recognition of the role that diet and exercise can play in functionally limiting symptom progression. This is an area for further study, as there is not a clear consensus guideline, but rather a body of case study work that suggests that appropriate physical activity can be an effective tool in managing disease progression. In one such study, side-alternating vibration training was used 3 times per week for 15 weeks. The results showed that, at 15 weeks, the patient had a 116 meter (70%) improvement to their 6MWT, which is significant compared with the results from the aforementioned LOTS study. Epidemiology The disease affects approximately 1 in 13,000. History
130_21	The disease is named after Joannes Cassianus Pompe, who characterized it in 1932. Pompe described accumulation of glycogen in muscle tissue in some cases of a previously unknown disorder. This accumulation was difficult to explain as the enzymes involved in the usual metabolism of glucose and glycogen were all present and functioning. The basis for the disease remained a puzzle until Christian de Duve's discovery of lysosomes in 1955 for which he won the Nobel Prize in 1974. His co-worker Henri G. Hers realised in 1965 that the deficiency of a lysosomal enzyme (alpha glucosidase) for the breakdown of glycogen could explain the symptoms of Pompe disease. This discovery led to establishing the concept of lysosomal storage diseases, of which 49 have been described (to date).
130_22	Despite recognizing the basis for the disease, treatment proved difficult. Administration of the enzyme lead to its uptake by the liver and not the muscle cells where it is needed. In the early 1990s Dutch scientists Arnold Reuser and Ans van der Ploeg were able to show that using alpha-glucosidase containing phosphorylated mannose residues purified from bovine testes increased the enzyme's activity in normal mouse muscles. Later in 1998, Dr. Yuan-Tsong Chen and colleagues at Duke University, using the enzyme produced in Chinese Hamster Ovary cells demonstrated for the first time that the enzyme can clear the glycogen and improved the muscle function in Pompe disease quail. The results of the work at Duke were impressive with one treated bird recovering to the point of being able to fly again.
130_23	This was followed by production of clinical grade alpha-glucosidase in Chinese hamster ovary (CHO) cells and in the milk of transgenic rabbits. This work eventually culminated in the start of clinical trials with the first clinical trial including 4 babies receiving enzyme from rabbit milk at Erasmus MC Sophia Children's Hospital and 3 babies receiving enzyme grown in CHO cells at Duke University in 1999.
130_24	The currently approved Myozyme is manufactured by Genzyme Corp. in Cambridge, Massachusetts. Its development was a complex process. Genzyme first partnered with Pharming Group NV who had managed to produce acid alpha-glucosidase from the milk of transgenic rabbits. They also partnered with a second group based at Duke University using Chinese hamster ovary cells. In 2001, Genzyme acquired Novazyme which was also working on this enzyme. Genzyme also had its own product (Myozyme) grown in CHO cells under development. In November 2001, Genzyme chief executive Henri Termeer organised a systematic comparison of the various potential drugs in a mouse model of Pompe disease. It was found that the Duke enzyme was the most efficacious, followed by Myozyme. However, due to easier manufacture of Myozyme, work on the other products was discontinued.
130_25	Funding for research in this field was in part provided by the Muscular Dystrophy Association and the Acid Maltase Deficiency Association in the US, and by the Association of Glycogen Storage Disorders in the UK, as well as the International Pompe Association. John Crowley became involved in the fund-raising efforts in 1998 after two of his children were diagnosed with Pompe. He joined the company Novazyme in 1999, which was working on enzyme replacement treatment for Pompe. Novazyme was sold to Genzyme in 2001 for over US$100 million. The 2010 film Extraordinary Measures is based on Crowley's search for a cure. As of 2019, many biomedical companies are developing Gene therapy in hopes of helping the body create alpha-glucosidase on its own. References External links GeneReview/NIH/UW entry on Glycogen Storage Disease Type II (Pompe Disease) Understanding Pompe Disease - US National Institute of Arthritis and Musculoskeletal and Skin Diseases
130_26	Autosomal recessive disorders Hepatology Inborn errors of carbohydrate metabolism Lysosomal storage diseases Rare diseases
131_0	The Dumaw Creek Site is an archaeological site designated 20OA5, located along Dumaw Creek northeast of Pentwater, Michigan, that was the location of a 17th-century village and cemetery. It is one of the youngest pre-historic sites in Michigan, dating to the terminal Late Woodland Period just prior to European contact. It was listed on the National Register of Historic Places in 1972. History The village that once stood at this location was likely inhabited in the early part of the 17th century (about 1605–1620, according to Quimby). The people occupying the village are thought to be the precursors of the modern Potawatomi people. It is likely that they were uprooted from this location soon after Samuel de Champlain's 1615 visit to the region, as warring tribes spilled into Michigan. Were they the Potawatomi people, they eventually made their way by the 1640s to the region around Sault Ste. Marie.
131_1	Meanwhile, white pine grew up in the plain where the village stood. The pine was lumbered in 1870-1880, after which the land was gradually converted to agricultural use, and was farmed as late as 1930. In the 1940s, oil was discovered in the area, and wells were installed. The Dumaw Creek site was originally discovered in 1915 by a farmer, Christoph "Carl" Schrumpf 1854–1949, who was pulling a stump from his field. Schrumpf discovered 18 skeletons and various artifacts in 1915–16. These artifacts were catalogued by the Museum of Anthropology at the University of Michigan in 1924, but the artifacts and records were not generally well known by archaeologists. Schrumpf eventually sold the collection to a private dealer, and they eventually made their way, sans any identifying data, to George I. Quimby at the Field Museum in 1959.
131_2	Quimby eventually tracked the source of the artifacts to the Dumaw Creek site, and in 1960-62 did some summer surface collection there. He also located other artifacts already dug from the site. Description The Dumaw Creek site is located on a sandy plain near Dumaw Creek, a tributary of the Pentwater River, at a site not easily accessible by canoe. The creek itself runs through a small valley about beneath the level of the plain. The site, covering , was used as a village and burial ground. The village is thought to have been a semi-permanent settlement with dome-shaped wigwams. The people hunted and fished, and grew corn and pumpkins. Artifacts Artifacts and features found at the site include burials, faunal remains, stone tools, bone tools, red ocher/mineral paint (used for ceremonial coloring), copper artifacts, shell artifacts, tobacco pipes, animal skins, and vegetal and textile remains.
131_3	At least nineteen, and perhaps as many as 55 skeletons were removed from burial sites in the Dumaw Creek site. The bodies were wrapped in furs and buried with stone implements and copper beads. The stone implements found include arrowheads, knives, and scraping tools of chipped flint and axes of a hard, granular stone. Quimby reports that 99% of the stone tools were small triangular points or arrowheads, aka Madison points, indicative of a Late Woodland period placement. There were also a considerable number of copper artifacts found at the site. Many were large bead-like tubes of copper known as hair pipes, which were worn as hair ornaments. There were also other, generally smaller copper beads, conical decorations, and copper plaques, as well as shell beads and pendants. Carved stone pipe bowls were also found, as well as animal skins and pottery.
131_4	The pottery is described by Quimby as grit-tempered vessels with round-bottomed, globular forms with broad orifices and slightly flaring rims. The rim edges are often scalloped or crimped. The vessel pictured below with a scalloped rim has been compared to the Moccasin Bluff Scalloped type from the Moccasin Bluff site on the St. Joseph River in southwestern Michigan. This pottery type is diagnostic of a Late Woodland period temporal placement, just before European contact. There was also a shell pendant with a "weeping eye" design as shown below. This motif has been seen in other Late Prehistoric sites in the Great Lakes area. References Buildings and structures in Oceana County, Michigan Archaeological sites on the National Register of Historic Places in Michigan National Register of Historic Places in Oceana County, Michigan Potawatomi Late Woodland period
132_0	Dag Ivar Wirén (15 October 1905 – 19 April 1986) was a Swedish composer. Life Wirén was born at Striberg near Nora. His father had a roller blind factory, and there were various musical activities in the family home; he took piano lessons, and was a student at the Karolinska school in Örebro, and played the bass drum and celesta in the town orchestra. Wirén studied at the Stockholm conservatory from 1926 to 1931, which gave him much exposure to music from all periods; hearing Arthur Honegger's oratorio King David in 1927 was an important experience.
132_1	In 1932 he won the state stipend and used the award money to continue his studies in Paris, where he lived from 1931 to 1934. While there, he studied composition under the Russian composer Leonid Sabaneyev, though he admitted later that his endless attendance of concerts, and not his tutoring with Sabaneyev, had the greater impact on his own work. In Paris he met Igor Stravinsky (as well as his compatriot Gösta Nystroem) and was exposed to the music of Stravinsky, Sergei Prokofiev and the French composers of Les Six.
132_2	In 1934, Wirén moved to Stockholm with his bride, the Irish cellist, Noel Franks, whom he had met in Paris; their daughter, Annika, was born in 1947. In 1937, the couple set up home permanently in Danderyd, just north of Stockholm. As well as composing, Wirén regularly played his main instrument, the piano, on Swedish Radio during the 1930s; he also devoted himself to chamber music in the 1930s and 40s; although he conducted in a recording of his Sinfonietta, he hated conducting. He was music critic at the "Svenska Morgonbladet" from 1938 to 1946, and in 1947 became vice-chairman of the Society of Swedish Composers. From 1948, he spent summers on the island of Björkö, in Stockholm's archipelago. He served as a member of the board of directors of the Royal Swedish Opera from 1962 to 1971. His TV ballet Den elaka drottningen (The Evil Queen) won the 1960 Prix Italia. He also wrote the music for the Swedish entry for the 1965 Eurovision Song Contest, Annorstädes vals (Absent Friend),
132_3	sung by Ingvar Wixell. He retired from composing in 1970, commenting, "One should stop in time, while one still has time to stop in time."
132_4	He died at Danderyd on 19 April 1986. Music Wirén's output, which ranges from serious to popular, is notable for its quality rather than quantity, and a number of his works were refused opus numbers or withdrawn. He once commented that his first desire was to entertain and please, and compose listener-friendly "modern" music. He was reluctant to write for the voice (in the 1965 Eurovision Song Contest entry, Alf Henrikson wrote the lyrics after Wirén had composed the music). Neoclassical pieces from Wirén's early Parisian period, including the Piano Trio (1933) and the Sinfonietta (1933–34), are melodically and rhythmically entertaining.
132_5	Upon his return to Sweden, he composed his first two symphonies and his most famous work, the Serenade for Strings (1937); the spirit of this serenade may also be found in the finale of his second symphony (1939). Wirén went on to compose five symphonies, concertos and other orchestral works, including music for the stage and film scores, as well as instrumental and chamber music, including a series of string quartets.
132_6	His musical style on return from Paris remained broadly neoclassical; melodic, energetic and with high spirits. Towards the mid-1940s Wirén became more serious in style, perhaps under the influence of Jean Sibelius. Wirén also developed a personal technique, first used in the third string quartet (1941–45), of gradual motivic transformation, avoiding repetition (metamorphosis technique). He pursued this approach in his third symphony (1944), where the first motif in the first movement, based on a step-wise Dorian mode progression, is transformed during the movement and then echoed in the last three movements. Motivic tautness also characterizes the deeply expressive fourth symphony (1952), as well as his rather cooler later works, including the fifth symphony (1964) and fifth string quartet (1970). Compositions
132_7	Symphonies Symphony No. 1, Op. 3 (1932), withdrawn and never performed Sinfonietta, Op. 7a (1933–34); Wirén's aborted attempt at a second symphony, subsequently refashioned into the Op. 7a Symphony No. 2, Op. 14 (1938–39) Symphony No. 3, Op. 20 (1943–44) Symphony No. 4, Op. 27 (1951–52) Symphony No. 5, Op. 38 (1963–64) Concert works Cello Concerto, Op. 10 (1936) Violin Concerto, Op. 23 (1946) Piano Concerto, Op. 26 (1950) Concertino for flute and small orchestra, Op. 44 (1972) Other orchestral Concert Overture 1, Op. 2 (1931) Two Orchestral Pieces, Op. 7b (1934) Serenade for Strings, Op. 11 (1937) Concert Overture 2, Op. 16 (1940) Little Suite, Op. 17 (1941) Romantic suite, Op. 22 (1943, rev. 1961) Lustspelsuvertyr, Op. 21 (1945) Divertimento, Op. 29 (1954–57) Triptych for small orchestra, Op. 33 (1958) Music for strings, Op. 40 (1966)
132_8	Chamber music String Quartet 1 String Quartet 2, Op. 9 (1935) String Quartet 3, Op. 18 (1941) String Quartet 4, Op. 28 (1952–53) String Quartet 5, Op. 41 (1970) Piano trio 1, Op. 6 (1933) Piano trio 2, Op. 36 (1961) Wind quintet, Op. 42 (1971) Quartet for flute, oboe, clarinet and cello, Op. 31 (1956) Sonatina for violin and piano, Op. 15 (1940) Sonatina for cello and piano 1, Op. 1 (1931) Sonatina for cello and piano 2, Op. 4 (1933) Miniature suite for cello and piano, Op. 8a (1934) Miniature suite for piano trio, Op. 8b (1934) Instrumental Theme with variations for piano, Op. 5 (1933) Small ironic pieces for piano, Op. 19 (1942–45) Sonatina for piano, Op. 25 (1950) Improvisations for piano, Op. 35 (1959) Little serenade for guitar, Op. 39 (1964) Little suite for piano, Op. 43 (1971) Choral Titania for women’s voices, words by Gustaf Fröding (1942) Three sea poems a capella, words by Karin Boye (1963)
132_9	Songs Livet och skrifterna ("En helig man"), words by Nils Ferlin (1934) Mitt trollslott står i skogens bryn, words by August Strindberg (1934) To your bed, Op. 13a, An autumn evening, Op. 13b, words by Erik Axel Karlfeldt (1938) Jungfru Maria and Malenavisorna, Op. 13 a-b (1938), words by Erik Axel Karlfeldt Annorstädes vals, words by Alf Henrikson (1965)
132_10	Works for the stage Ballet Oscarian ball, Op. 24, after Gustaf Fröding’s "Balen" (1948–49), (Royal Stockholm Opera, 1950, choreography by Birgit Cullberg) Radio operetta, Gult, rött och blått, libretto by Georg Eliasson (Swedish Radio, 1940) Radio operetta, Den glada patiencen, libretto by Georg Eliasson (Swedish Radio, 1941) Incidental music for Madame Bovary, (Gustave Flaubert), Blancheteatern 1939 Stage music for Amorina (Carl Jonas Love Almqvist), Kungliga Dramatiska Teatern, 1951 Music for The Merchant of Venice, William Shakespeare (1943), Kungliga Dramatiska Teatern Incidental music for Romeo and Juliet (William Shakespeare), Kungliga Dramatiska Teatern, 1953 Stage music for Gudens hustru, (Vilhelm Moberg), Kungliga Dramatiska Teatern, 1954 Music for A midsummer night’s dream, Kungliga Dramatiska Teatern, 1955 Music for The Queen's Tiara (Carl Jonas Love Almqvist), Kungliga Dramatiska Teatern, 1957 Ballet Take your place on stage
132_11	Ballet, Den elaka drottningen, Sveriges Television 1960, choreography by Birgit Cullberg Stage music for Hamlet (Shakespeare), Kungliga Dramatiska Teatern, 1960 Stage music for King John (Shakespeare), Kungliga Dramatiska Teatern, 1961
132_12	Film music Man glömmer ingenting (One Forgets Nothing) (1942) Oss tjuvar emellan eller En burk ananas (1945) Grönt guld (1949) Only a Mother (1949) Miss Julie (1951) A Lesson in Love (1954) Wild Birds (1955) The Phantom Carriage (1958) De sista stegen (A matter of morals) (1961) Recordings As conductor Wirén recorded his Sinfonietta in May 1948 for Cupol, reissued on Phono Suecia PSCD 79, 1995. References External links A list of Wirén autographs with some information about dates Broadcast from 1960 of Wirén's Violin Concerto Op. 23 (British Library)
132_13	1905 births 1986 deaths 20th-century classical composers Litteris et Artibus recipients Swedish opera composers Male opera composers Royal College of Music, Stockholm alumni People from Nora Municipality Swedish classical composers Swedish male classical composers Swedish classical pianists Male classical pianists Swedish film score composers Male film score composers Swedish music critics 20th-century classical pianists 20th-century Swedish male musicians 20th-century Swedish musicians
133_0	Jason Eli Becker (born July 22, 1969) is an American virtuoso musician, songwriter and composer. At the age of 16, he became part of the Shrapnel Records-produced duo Cacophony with his friend Marty Friedman, and they released two albums, Speed Metal Symphony (1987) and Go Off! (1988). Since the dissolution of Cacophony in 1989, Becker has undertaken a solo career, releasing seven albums since his 1988 debut Perpetual Burn. He later joined David Lee Roth's band and recorded one album with him, A Little Ain't Enough. Becker's performing career was cut short by amyotrophic lateral sclerosis (ALS), which he was diagnosed with in 1989. By 1996, Becker had lost the ability to speak, and he now communicates with his eyes via a system developed by his father. He continues to compose with the aid of a computer and has since released Collection in 2008 and Triumphant Hearts in 2018, as well as various compilations. Biography and career
133_1	Hometown, birthplace, school and early years Becker was born and raised in Richmond, California, by his parents, Gary and Patricia (Heffley) Becker. He was born in Richmond Hospital on 23rd Street in 1969. His maternal grandfather was actor Wayne Heffley. Becker graduated from Kennedy High School where he performed Yngwie Malmsteen's "Black Star" with his band at a talent show. While still in high school, Becker was introduced to Marty Friedman. He was exposed to the guitar at an early age because both his father and his uncle were guitar players. He absorbed all kinds of music from around the world and melded different aspects of each style into his playing. He cited Bob Dylan, Eric Clapton, Jimi Hendrix and Eddie Van Halen as early influences.
133_2	Cacophony and solo career Becker started out playing alongside Marty Friedman in the Mike Varney-produced duo, Cacophony. Together, they put out an album, and toured Japan and the U.S. While they never went mainstream in the U.S., they proved popular enough in Europe to sell out almost every performance. In 1990, Friedman left to join Megadeth and Becker began to pursue a solo career, having released his first solo album titled Perpetual Burn in 1988. He has since released the albums Perspective and Collection, as well as two albums of demos, entitled The Raspberry Jams and The Blackberry Jams.
133_3	Amyotrophic lateral sclerosis diagnosis In 1989, Becker joined David Lee Roth's solo band to work on his third album A Little Ain't Enough, replacing Steve Vai, who left the band to record and tour with Whitesnake. While preparing for the album, Becker began to feel what he called a "lazy limp" in his left leg. He was soon diagnosed with amyotrophic lateral sclerosis (ALS; Lou Gehrig's disease) and was given three to five years to live. He finished the recording using lighter gauge guitar strings and other techniques, which made it easier for him to play with his weakening hands. Although he managed to finish the album, he did not join the supporting tour due to his inability to perform on stage; former Lizzy Borden guitarist Joe Holmes took Becker's place on tour.
133_4	His ALS gradually robbed him of his ability to play guitar, to walk, and eventually his ability to speak. He now communicates with his eyes via a system developed by his father. Due to the nature of the disease, he remains mentally sharp and, with the aid of a computer, continues composing. In the back of the Perspective CD case, Becker states "I have Amyotrophic Lateral Sclerosis. It has crippled my body and speech, but not my mind." His medical condition has remained stable since 1997. In 2003, Becker posted on his website that he was feeling better and had gained some weight, while the folder for his 2008 album Collection also mentions an upcoming book.
133_5	Perspective and Raspberry Jams In 1996, Becker released an album entitled Perspective, an instrumental album composed by him (with the exception of Bob Dylan's song "Meet Me in the Morning"). The writing of the music had been started before ALS completely crippled his abilities. By using guitar, and, later, when he was unable to use both hands, a keyboard, he continued to compose while his disease worsened. However, when Becker could no longer physically play even a keyboard, his friend and music producer Mike Bemesderfer helped him with a music-composing computer program that reads movements of his head and eyes, enabling Becker to continue to compose after he lost control of the rest of his body. Several years later, Becker released Raspberry Jams (1999) and Blackberry Jams (2003); the first contained various unreleased demo-tracks, and the latter contained demo-tracks and alternate versions of songs that were later reworked and published into other albums.
133_6	Two tribute albums to Jason Becker have been issued. Respectively entitled Warmth in the Wilderness I and Warmth in the Wilderness II, they feature guitarists such as Steve Vai, Paul Gilbert, Marty Friedman, Joe Becker, Rusty Cooley, and Mattias Eklundh. The album profits were sent to Becker to help him with his medical finances. Collection On November 4, 2008, Shrapnel Records released a new Jason Becker album entitled Collection. The album includes three new songs in addition to some older recordings (some never before released) and features Marty Friedman, Greg Howe, Joe Satriani, Michael Lee Firkins, Steve Vai, and Steve Hunter. Boy Meets Guitar On August 20, 2010, Becker declared that he was considering releasing an album of music he recorded when he was in his teenage years. He has since released this album, titled Boy Meets Guitar, in 2012.
133_7	Triumphant Hearts In October 2016, Becker launched a campaign to fund an album, which was initially estimated for release in July 2017. The campaign raised more than $100,000. The album, Triumphant Hearts was released on December 7, 2018.
133_8	Musical style
133_9	Considered a virtuoso guitarist and one of the top players of his time, Becker studied the works of violinist Niccolò Paganini and was a playing partner with Marty Friedman. He later arranged Paganini's 5th Caprice, performing it during an instructional guitar video. Becker's compositions often include high speed scalar and arpeggio passages—trademarks of his shred style of guitar playing. Often incorporating advanced techniques such as sweep picking, alternate picking, artificial harmonic accenting, tapping and hybrid picking; he was among the leaders of the field during the technical shred guitar and neoclassical metal trend of the mid to late 1980s and is still respected and honored by his musician peers today. The song "Serrana" appearing in the album Perspective, is an example of his sweep-picking skills. He demonstrated the arpeggio sequence during a clinic at the Atlanta Institute of Music. A video of this performance first appeared on his Hot Licks guitar instructional video.
133_10	Influence on other guitarists Becker's playing has proven to be influential on countless guitarists, including Nita Strauss, Guthrie Govan, Herman Li, Chris Broderick, and Daniel Mongrain, among others. Media Becker has appeared on many magazine covers throughout his career, including the February 1991 issue of Guitar for the Practicing Musician along with Jim Martin of Faith No More, the June 1991 issue of Young Guitar, and the July 2012 issue of Guitar Player, among others. He was also extensively interviewed for the 2017 book, Shredders!: The Oral History Of Speed Guitar (And More), by author Greg Prato.
133_11	Equipment Becker's first guitar was a Franciscan acoustic. Prior to joining Cacophony in 1987, Becker worked his way through the Franciscan, a Takamine acoustic, a Fender Musicmaster, and finally, a black "Dan Smith" era Fender Stratocaster (likely a 1982 or 1983 model) with a DiMarzio Steve Morse humbucker in the bridge (seen in the "Black Star" video, circa 1986). On his message board, Becker said "I recorded SMS [Speed Metal Symphony] with my Strat." For his next guitar, Becker said on his message board "Mike Varney got Hurricane to endorse us after we recorded Speed Metal Symphony. I liked how they were like Strats, only beefier." On Perpetual Burn, Becker said on his message board: "I used my white and black Hurricane guitar for everything. For clean tone I went direct. For dirty tone I used a 100 Watt Marshall with a Boss Super Overdrive pedal."
133_12	The Moridira Hurricane guitar he used is called a Limited Edition LTD.2 model, believed to be made in Japan. (It is not a Hurricane EX series, which appears to be lower quality.) His LTD.2 was a "strat copy", but with a HSS pickup setup, a 24-fret rosewood fingerboard, and unique Floyd Rose where you do not have to cut the strings. For pickups, Becker said on his message board: "I just used the stock pickups it came with." The pickups were Japanese-made pickups. While Becker is pictured with a blue Hurricane guitar on the cover of Perpetual Burn, he did not use this blue guitar on the album. Differences on this guitar from his first Hurricane include DiMarzio pickups, a maple fretboard, and 24 frets. (Marty Friedman recorded the whammy parts of his song "Dragon Mistress" using Becker's blue Hurricane, one of the rare times Marty has recorded whammy work.)
133_13	For the second Cacophony album, Becker switched over to Carvin gear, utilizing 2 DC Series models, one in a trans blue finish with flamed maple top, and another one in a solid burgundy finish (This is the guitar seen in the famous "Yo Yo" video from the 1989 Japan tour with Cacophony). Both have double cutaway bodies, Kahler locking tremolo systems, six in line machine heads and two Carvin humbucker pickups. He used these up until he was diagnosed with ALS in 1989.
133_14	During the sessions for A Little Ain't Enough, Becker used various Carvin, Ibanez, ESP and Valley Arts guitars, as well as a Les Paul on some tracks and a Gibson acoustic for select things. Becker has also been pictured with a few Hamer superstrats as well. From 1989–1991, Becker used various guitars, most notably a Peavey custom model with the numbered fretboard markers. Also used were an Ibanez Custom Shop guitar (probably based on an RG), a custom from Performance Guitars, a couple of various unknown Strat style guitars and a black Hurricane with three single coil pickups. Becker has allowed Paradise Guitars USA to release a Jason Becker signature guitar. It is similar in appearance to the numbered Peavey, but with a different headstock shape.
133_15	Becker used various types of amplifiers in his music. Before joining Cacophony, he used a small Peavey Studio Pro 40 with the older style Peavey vertical silver stripes on the grille cloth. During his early days Becker was also seen with a red Marshall JCM800 head and 4x12 cabinet. For the first Cacophony album, Becker used an ADAMP1 preamp. He recorded Perpetual Burn with a borrowed 1970s Marshall half stack and a BOSS Super Overdrive and Cacophony's second album was recorded with a Carvin X100B stack. For the David Lee Roth album A Little Ain't Enough, Becker used "eight different Marshall amps." He also used the SX300H head at some point during that era. After Cacophony, Becker used various amps, including a "Fender M80", an unknown Marshall amp, an ADA Preamp and possibly the aforementioned Peavey combo. Becker typically used Dean Markley and SIT strings.
133_16	Paradise Guitar In 2008 Paradise Guitars worked with Becker to design a Jason Becker signature guitar. The design is based on the Peavey with colored number fret inlays. Features include an alder body, maple neck with steel 2-way truss rod, maple 16" radius fingerboard, 24 jumbo thin frets with colored number fret marker inlays, black Floyd Rose Pro Style floating Tremolo with Floyd Rose Tremolo stop, Sperzel red satin tuners, 14-degree tilt-back headstock with black Paradise logo and matching tremolo and electronics plates. The pickups are DiMarzio pickups; a PAF Pro-Custom in the neck colored yellow and red, a DP116 HS-2 in the middle colored green, and a Tone Zone-Custom in the bridge colored pink and blue. These colored pickups complement the colored inlays and seem to give the guitar a rainbow effect. There is also a red five-way switch and purple 1–11 volume knob.
133_17	Kiesel/Carvin Tribute Guitars In 2012, Carvin worked with Becker to design the JB200C Jason Becker Tribute, a guitar that is modeled after the original DC200 guitar he used toward the latter part of his career. The guitar features an Alder body with flamed maple top, maple neck with a flamed maple fingerboard, a Floyd Rose tremolo, jumbo frets, 2 humbuckers, active electronics, and comes standard in a transparent blue finish.
133_18	In 2015, Kiesel Guitars, which took over Carvin's guitar manufacturing, worked with Becker to release a second tribute model, called the JB24 "Numbers" guitar. It is the third incarnation of his "Numbers" guitar, previously released by Peavey and Paradise guitars, and is also one of the first Carvin guitars to feature a 24-fret bolt-on neck, alongside the GH24 Greg Howe signature model released in the same year. It features a tung-oiled ash body, maple neck and fingerboard, colored number inlays, stainless steel frets, and 3 custom-colored Seymour Duncan pickups (Perpetual Burn in the bridge, Vintage Hot Stack in the middle, and Jazz in the neck).
133_19	For the official launch of Becker's signature Seymour Duncan Perpetual Burn Humbucker and the Carvin JB24 numbers guitar, Danny Young was chosen as the guitarist for both performances due to stylistic resemblance to Jason Becker and Niccolo Paganini. The Perpetual Burn performance was played on the Carvin JB200C. These performances led to the widespread notability of Danny Young's virtuosity in the guitar community and an authority in the tone and playing style of Jason Becker. Documentary film A feature-length documentary film about the life of Jason Becker entitled Jason Becker: Not Dead Yet, was released in 2012. The film includes interviews with Becker, his family and friends, and the various musicians he has worked with, including Marty Friedman, Steve Vai, Joe Satriani, Richie Kotzen, and Steve Hunter. The film was generally well received by fans and critics and won many awards. Clinics Atlanta Institute of Music Year: 1989 Gear: Carvin DC200, Marshall Amp, Alesis quadraverb
133_20	Japan Clinic Year: 1989 Gear: Carvin DC200 Discography Cacophony Speed Metal Symphony (1987) Go Off! (1988) Marty Friedman Dragon's Kiss (1988) Inferno (2014) Solo Perpetual Burn (1988) Perspective (1996) The Raspberry Jams (1999) The Blackberry Jams (2003) Collection (2008) Boy Meets Guitar, Vol. 1 of the Youngster Tapes (2012) Triumphant Hearts (2018) David Lee Roth A Little Ain't Enough (1991) Joe Becker Short Stories (2005) Other works Richie Kotzen (1989, producer) Daydream by the Hudson on Steve Hunter's 2013 The Manhattan Blues Project Compilations Guitar Masters, 1989, Roadrunner Records Metal Guitars – High Voltage Instrumentals,1998, Disky Communications Shrapnel's Super Shredders: Neoclassical,2009, Shrapnel This is Shredding, Vol. 1, 2009, Shrapnel This is Shredding, Vol. 2, 2009, Shrapnel Tribute
133_21	Warmth in the Wilderness: A Tribute to Jason Becker, 2001, Lion Music Warmth in Wilderness 2: Tribute Jason Becker, 2002, Lion Music Jason Becker's Not Dead Yet! (Live in Haarlem), 2012, Primal Events Instructional Hot Licks – The Legendary Guitar of Jason Becker In The Style Of Jason Becker, feat. Max Dible. DC Music School Films Jason Becker: Not Dead Yet (2012) One Track Heart: The Story of Krishna Das (2012) References External links Jason Becker's official website Becker's MySpace page Shrapnel Records official website Documentary film website Paralyzed guitarist continues to produce music \| Abc7news.com 1969 births Living people American heavy metal guitarists People with motor neuron disease Lead guitarists Musicians from Richmond, California Guitarists from California American male guitarists 20th-century American guitarists Cacophony (band) members 20th-century American male musicians Shrapnel Records artists
134_0	Amy Johnson (born 1 July 1903 – disappeared 5 January 1941) was a pioneering English pilot who was the first woman to fly solo from London to Australia. Flying solo or with her husband, Jim Mollison, she set many long-distance records during the 1930s. She flew in the Second World War as a part of the Air Transport Auxiliary and disappeared during a ferry flight. The cause of her death has been a subject of discussion over many years. Early life Born in 1903 in Kingston upon Hull, East Riding of Yorkshire, Amy Johnson was the daughter of Amy Hodge, granddaughter of William Hodge, a Mayor of Hull, and John William Johnson whose family were fish merchants in the firm of Andrew Johnson, Knudtzon and Company. She was the eldest of three sisters, the next in age being Irene who was a year younger.
134_1	Johnson was educated at Boulevard Municipal Secondary School (later Kingston High School) and the University of Sheffield, where she graduated with a Bachelor of Arts degree in economics. She then worked in London as secretary to a solicitor, William Charles Crocker. She was introduced to flying as a hobby, gaining an aviator's certificate, No. 8662, on 28 January 1929, and a pilot's "A" Licence, No. 1979, on 6 July 1929, both at the London Aeroplane Club under the tutelage of Captain Valentine Baker. In that same year, she became the first British woman to obtain a ground engineer's "C" licence. Johnson was a friend and collaborator of Fred Slingsby whose Yorkshire based company, Slingsby Aviation of Kirbymoorside, North Yorkshire became the UK's most famous glider manufacturer. Slingsby helped found Yorkshire Gliding Club at Sutton Bank and during the 1930s she was an early member and trainee. Aviation
134_2	Johnson obtained the funds for her first aircraft from her father, who was always one of her strongest supporters, and Lord Wakefield. She purchased a secondhand de Havilland DH.60 Gipsy Moth G-AAAH and named it Jason after her father's business trade mark. Johnson achieved worldwide recognition when, in 1930, she became the first woman to fly solo from England to Australia. Flying G-AAAH Jason, she left Croydon Airport, Surrey, on 5 May and landed at Darwin, Northern Territory on 24 May . Six days later she damaged her aircraft while landing downwind at Brisbane airport and flew to Sydney with Captain Frank Follett while her plane was repaired. Jason was later flown to Mascot, Sydney, by Captain Lester Brain. G-AAAH "Jason" is now on permanent display in the Flight Gallery of the Science Museum in London.
134_3	She received the Harmon Trophy as well as a CBE in George V's 1930 Birthday Honours in recognition of this achievement, and was also honoured with the No. 1 civil pilot's licence under Australia's 1921 Air Navigation Regulations. Johnson next obtained a de Havilland DH.80 Puss Moth G-AAZV which she named Jason II. In July 1931, she and co-pilot Jack Humphreys became the first people to fly from London to Moscow in one day, completing the journey in approximately 21 hours. From there, they continued across Siberia and on to Tokyo, setting a record time for Britain to Japan. In 1932, Johnson married Scottish pilot Jim Mollison, who had proposed to her during a flight together some eight hours after they had first met. In July 1932, Johnson set a solo record for the flight from London to Cape Town, South Africa in Puss Moth G-ACAB, named Desert Cloud, breaking her new husband's record. De Havilland Co and Castrol Oil featured this flight in advertising campaigns.
134_4	In July 1933, Johnson together with Mollison flew the G-ACCV, named "Seafarer," a de Havilland DH.84 Dragon I nonstop from Pendine Sands, South Wales, heading to Floyd Bennett Field in Brooklyn, New York. The aim was to take “Seafarer” to the starting point for the Mollison's attempt at achieving a world record distance flying non-stop from New York to Baghdad. Running low on fuel and now flying in the dark of night, the pair made the decision to land short of New York. Spotting the lights of Bridgeport Municipal Airport (now Sikorsky Memorial Airport) in Stratford, Connecticut they circled it five times before crash landing some distance outside the field in a drainage ditch. Both were thrown from the aircraft but suffered only cuts and gashes. After recuperating, the pair were feted by New York society and received a ticker tape parade down Wall Street.
134_5	The Mollisons also flew, in record time, from Britain to India in 1934 in G-ACSP, named "Black Magic", a de Havilland DH.88 Comet as part of the Britain to Australia MacRobertson Air Race, but were forced to retire from the race at Allahabad because of engine trouble. In September 1934, Johnson (under her married name of Mollison) became the youngest President of the Women's Engineering Society, having been vice-president since 1934. She was active in the society until her death. On 4 May 1936, Johnson made her last record-breaking flight, starting from Gravesend Airport & regaining her Britain to South Africa record in G-ADZO, a Percival Gull Six. The same year she was awarded the Gold Medal of the Royal Aero Club. In 1938, Johnson overturned her glider when landing after a display at Walsall Aerodrome in England, but was not seriously hurt. The same year, she divorced Mollison. Soon afterwards, she reverted to her maiden name.
134_6	Johnson began to explore other ways to make a living through business ventures, journalism and fashion. She modelled clothes for the designer Elsa Schiaparelli and created her a travelling bag sold under her own name. In 1939 Johnson found work flying with the Portsmouth, Southsea and Isle of Wight Aviation Company, piloting short flights across the Solent and flying as a target for searchlight batteries and anti-aircraft gunners to practice on. The company’s aircraft were taken over by the Air Ministry in March 1940 and Johnson was served notice of redundancy alongside all other pilots in the company as all the aircraft were requisitioned for the war effort. She received a week's pay and a further four weeks pay of £40 as a redundancy package.
134_7	Second World War Two months later in 1940, during the Second World War, Johnson joined the newly formed Air Transport Auxiliary (ATA), which transported Royal Air Force aircraft around the country. She rose to First Officer under the command of her friend and fellow pilot Pauline Gower. Her former husband also flew for the ATA throughout the war. Johnson described a typical day in her life in the ATA in a humorous article (published posthumously in 1941) for The Woman Engineer journal. Disappearance Writing a last letter to her friend Caroline Haslett, on New Years Day 1941, "I hope the gods will watch over you this year, and I wish you the best of luck (the only useful thing not yet taxed!)." On 5 January 1941, while flying an Airspeed Oxford for the ATA from Prestwick via RAF Squires Gate to RAF Kidlington near Oxford, Johnson went off course in adverse weather conditions. Reportedly out of fuel, she bailed out as her aircraft crashed into the Thames Estuary near Herne Bay.
134_8	A convoy of wartime vessels in the Thames Estuary spotted Johnson's parachute coming down and saw her alive in the water, calling for help. Conditions were poor – there was a heavy sea and a strong tide, snow was falling and it was intensely cold. Lt Cmdr Walter Fletcher, the Captain of HMS Haslemere, navigated his ship to attempt a rescue. The crew of the vessel threw ropes out to Johnson but she was unable to reach them and was lost under the ship. A number of witnesses believed there was a second body in the water. Fletcher dived in and swam out to this, rested on it for a few minutes then let go. When the lifeboat reached him he was unconscious and as a result of the intense cold he died in hospital days later. Johnson's watertight flying bag, her log book and cheque book later washed up and recovered near the crash site.
134_9	A memorial service was held for Johnson in the church of St Martin-in-the-Fields on 14 January 1941. Walter Fletcher was posthumously awarded the Albert Medal in May 1941. Disputed circumstances In 1999, it was reported that Johnson's death may have been caused by friendly fire. Tom Mitchell, from Crowborough, Sussex, claimed to have shot Johnson's aircraft down when she twice failed to give the correct identification code during the flight. Mitchell explained how the aircraft was sighted and contacted by radio. A request was made for the signal. She gave the wrong one twice. "Sixteen rounds of shells were fired and the plane dived into the Thames Estuary. We all thought it was an enemy plane until the next day when we read the papers and discovered it was Amy. The officers told us never to tell anyone what happened."
134_10	In 2016, Alec Gill, a historian, claimed that the son of a ship's crew member stated that Johnson had died because she was sucked into the blades of the ship's propellers; the crewman did not observe this to occur, but believes it is true. As a member of the ATA with no known grave – her body was never recovered – Johnson is commemorated (under the name Amy V. Johnson) by the Commonwealth War Graves Commission on the Air Forces Memorial at Runnymede. Honours and tributes In June 1930, Johnson's flight to Australia was the subject of a contemporary popular song, "Amy, Wonderful Amy", composed by Horatio Nicholls and recorded by Harry Bidgood, Jack Hylton, Arthur Lally, Arthur Rosebery and Debroy Somers. She was also the guest of honour at the opening of the first Butlins holiday camp, in Skegness in 1936. From 1935 to 1937, Johnson was the President of the Women's Engineering Society.
134_11	A collection of Amy Johnson souvenirs and mementos was donated by her father to Sewerby Hall in 1958. The hall now houses a room dedicated to Amy Johnson in its museum. In 1974, Harry Ibbetson's statue of Amy Johnson was unveiled in Prospect Street, Hull where a girls' school was named after her (the school closed in 2004). In 2016 new statues of Johnson were unveiled to commemorate the 75th anniversary of her death. The first, on 17 September, was at Herne Bay, close to the site she was last seen alive, and the second, on 30 September, was unveiled by Maureen Lipman near Hawthorne Avenue, Hull, close to Johnson's childhood home.
134_12	In 2017 The Guardian listed the Amy Johnson bronze as one of the "best female statues in Britain". A blue plaque commemorates Johnson at Vernon Court, Hendon Way, in Childs Hill, London NW2. She is commemorated with a green plaque on The Avenues, Kingston upon Hull. She is commemorated with another blue plaque in Princes Risborough where she lived for a year.
134_13	Buildings named in Johnson's honour include "Amy Johnson Building" housing the department of Automatic Control and Systems Engineering at the University of Sheffield. "Amy Johnson Primary School" situated on Mollison Drive on the Roundshaw Estate, Wallington, Surrey, which is built on the former runway site of Croydon Airport. "The Hawthornes @ Amy Johnson" in Hull, a major housing development by Keepmoat Homes on the site of the former Amy Johnson School. Amy Johnson Comet Restoration Centre at Derby Airfield, where the Mollison's DH.88 Comet Black Magic is being restored to flying condition. Amy Johnson House in Cherry Orchard Road, Croydon was named for her; built in the 20th-century it was demolished in the mid 2010s.
134_14	Other tributes to Johnson include a KLM McDonnell-Douglas MD-11, and after that aircraft was retired, a Norwegian Air UK Boeing 787-9, named in her honour, and "Amy's Restaurant and Bar" at the Hilton hotels at both London Gatwick and Stansted airports are named after her. "Amy Johnson Avenue" is a main road running northwards from Tiger Brennan Drive, Winnellie, to McMillans Rd, Karama, In Darwin, Australia. "Amy Johnson Way" is a road linking commercial premises in Blackpool, Lancashire, UK, adjacent to Blackpool Airport. It is also the name of a road in Clifton Moor, York. "Johnson Road" is one of the roads built on the site of the former Heston Aerodrome in west London.
134_15	In 2011 the Royal Aeronautical Society established the annual Amy Johnson Named Lecture to celebrate a century of women in flight and to honour Britain's most famous woman aviator. Carolyn McCall, Chief Executive of EasyJet, delivered the Inaugural Lecture on 6 July 2011 at the Society's headquarters in London. The Lecture is held on or close to 6 July every year to mark the date in 1929 when Amy Johnson was awarded her pilot's licence. Over a six-month period, inmates of Hull Prison built a full-size model of the Gipsy Moth aircraft used by Johnson to fly solo from Britain to Australia. In February 2017 this went on public display at Hull Paragon Interchange.

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