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Such reduced ATP concentrations are correlated with reduced poly(ADP\u2010ribose) synthetase (polyADPRT; EC 2.4.2.30) activity; this reduces the breakdown of nicotinamide\u2010adenine dinucleotide coenzymes and thus explains their normal intracellular concentrations. Since reductions in poly(ADP\u2010ribose) synthetase activity reduce DNA repair, alterations in DNA could accumulate even in non\u2010multiplying cells such as neurons, especially in the continuously active \u2018respiratory centre\u2019. Our Lesch\u2010Nyhan patients suffered respiratory deaths between 15 and 20 years of age.<\/jats:p>","DOI":"10.1007\/bf02436765","type":"journal-article","created":{"date-parts":[[2006,7,27]],"date-time":"2006-07-27T10:42:28Z","timestamp":1153996948000},"page":"737-747","source":"Crossref","is-referenced-by-count":15,"title":["Lesch\u2010Nyhan syndrome and its pathogenesis: Normal nicotinamide\u2010adenine dinucleotide but reduced ATP concentrations that correlate with reduced poly (ADP\u2010ribose) synthetase activity in HPRT\u2010deficient lymphoblasts"],"prefix":"10.1002","volume":"18","author":[{"given":"G. M.","family":"McCreanor","sequence":"first","affiliation":[{"name":"Division of Inherited Metabolic Diseases MRC Clinical Research Centre and Northwick Park Hospital  Harrow Middlesex"},{"name":"Department of Chemical Pathology Royal Free Hospital  Pond Street London NW3 2QG UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"R. 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