{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,11,7]],"date-time":"2025-11-07T18:51:49Z","timestamp":1762541509455},"reference-count":82,"publisher":"Canadian Science Publishing","issue":"1","license":[{"start":{"date-parts":[[2006,2,1]],"date-time":"2006-02-01T00:00:00Z","timestamp":1138752000000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/www.nrcresearchpress.com\/page\/about\/CorporateTextAndDataMining"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Appl. Physiol. Nutr. Metab."],"published-print":{"date-parts":[[2006,2,1]]},"abstract":"<jats:p>Exercise physiologists are interested in metabolic myopathies because they demonstrate how knocking out a component of a specific biochemical pathway can alter cellular metabolism. McArdle's disease (myophosphorylase deficiency) has often been studied in exercise physiology to demonstrate the influence of removing the major anaerobic energy supply to skeletal muscle. Studies of patients with McArdle's disease have shown the increased reliance on blood-borne fuels, the importance of glycogen to maximal aerobic capacity, and the use of nutritional strategies to bypass metabolic defects. Myoadenylate deaminase deficiency is the most common metabolic enzyme deficiency in human skeletal muscle. It is usually compensated for endogenously and does not have a major influence on high-energy power output. Nutritional interventions such as carbohydrate loading and carbohydrate supplementation during exercise are essential components of therapy for patients with fatty acid oxidation defects. Cases of mitochondrial myopathies illustrate the importance of peripheral oxygen extraction for maximal aerobic capacity and show how both exercise and nutritional interventions can partially compensate for these mutations. In summary, metabolic myopathies provide important insights into regulatory and nutritional aspects of the major biochemical pathways of intermediary metabolism in human skeletal muscle. Key words: myoadenylate deaminase deficiency, MELAS syndrome, McArdle's disease, mitochondrial disease, inborn errors of metabolism.<\/jats:p>","DOI":"10.1139\/h05-008","type":"journal-article","created":{"date-parts":[[2006,3,5]],"date-time":"2006-03-05T19:13:06Z","timestamp":1141585986000},"page":"21-30","source":"Crossref","is-referenced-by-count":18,"title":["What can metabolic myopathies teach us about exercise physiology?"],"prefix":"10.1139","volume":"31","author":[{"given":"Mark A","family":"Tarnopolsky","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"155","reference":[{"key":"p_1\/p_1_1","doi-asserted-by":"crossref","first-page":"1248","DOI":"10.1016\/S0735-1097(00)00850-0","volume":"36","author":"Anderson J.L.","year":"2000","journal-title":"J. Am. Coll. Cardiol."},{"key":"p_2\/p_2_1","first-page":"741","volume":"43","author":"Barbiroli B.","year":"1997","journal-title":"Cell Mol. Biol. (Noisy-Le-Grand)"},{"key":"p_3\/p_3_1","doi-asserted-by":"crossref","first-page":"130","DOI":"10.1016\/S0014-5793(99)01773-1","volume":"466","author":"Bender E.","year":"2000","journal-title":"FEBS Lett."},{"key":"p_4\/p_4_1","doi-asserted-by":"crossref","first-page":"187","DOI":"10.1097\/01.wnf.0000137862.67131.bf","volume":"27","author":"Berbel-Garcia A.","year":"2004","journal-title":"Clin. Neuropharmacol."},{"key":"p_5\/p_5_1","doi-asserted-by":"crossref","first-page":"218","DOI":"10.3109\/00365516709090629","volume":"19","author":"Bergstrom J.","year":"1967","journal-title":"Scand. J. Clin. Lab. Invest."},{"key":"p_6\/p_6_1","doi-asserted-by":"crossref","first-page":"140","DOI":"10.1111\/j.1748-1716.1967.tb03720.x","volume":"71","author":"Bergstrom J.","year":"1967","journal-title":"Acta Physiol. Scand."},{"key":"p_7\/p_7_1","doi-asserted-by":"crossref","first-page":"1406","DOI":"10.1212\/01.WNL.0000033795.17156.00","volume":"59","author":"Bernier F.P.","year":"2002","journal-title":"Neurology"},{"key":"p_8\/p_8_1","doi-asserted-by":"crossref","first-page":"1674","DOI":"10.1002\/(SICI)1097-4598(199912)22:12<1674::AID-MUS9>3.0.CO;2-0","volume":"22","author":"Blazev R.","year":"1999","journal-title":"Muscle Nerve"},{"key":"p_9\/p_9_1","doi-asserted-by":"crossref","first-page":"1299","DOI":"10.1002\/(SICI)1097-4598(199909)22:9<1299::AID-MUS24>3.0.CO;2-4","volume":"22","author":"Borchert A.","year":"1999","journal-title":"Muscle Nerve"},{"key":"p_10\/p_10_1","doi-asserted-by":"crossref","first-page":"E898","DOI":"10.1152\/ajpendo.2001.280.6.E898","volume":"280","author":"Carter S.L.","year":"2001","journal-title":"Am. J. Physiol. Endocrinol. Metab."},{"key":"p_11\/p_11_1","doi-asserted-by":"crossref","first-page":"7","DOI":"10.1016\/0925-4439(95)00003-M","volume":"1271","author":"Chance B.","year":"1995","journal-title":"Biochim. Biophys. Acta"},{"key":"p_12\/p_12_1","doi-asserted-by":"crossref","first-page":"212","DOI":"10.1159\/000117445","volume":"37","author":"Chen R.S.","year":"1997","journal-title":"Eur. Neurol."},{"key":"p_13\/p_13_1","doi-asserted-by":"crossref","first-page":"182","DOI":"10.1378\/chest.108.1.182","volume":"108","author":"Dandurand R.J.","year":"1995","journal-title":"Chest"},{"key":"p_14\/p_14_1","doi-asserted-by":"crossref","first-page":"999","DOI":"10.1152\/japplphysiol.01118.2002","volume":"95","author":"Dawson K.D.","year":"2003","journal-title":"J. Appl. Physiol."},{"key":"p_15\/p_15_1","doi-asserted-by":"crossref","first-page":"37","DOI":"10.1001\/archneur.62.1.37","volume":"62","author":"Deschauer M.","year":"2005","journal-title":"Arch. Neurol."},{"key":"p_16\/p_16_1","first-page":"123","volume":"17","author":"Dimauro S.","year":"1977","journal-title":"Adv. Neurol."},{"key":"p_17\/p_17_1","doi-asserted-by":"crossref","first-page":"199","DOI":"10.1016\/S0005-2728(98)00113-3","volume":"1366","author":"Dimauro S.","year":"1998","journal-title":"Biochim. Biophys. Acta"},{"key":"p_18\/p_18_1","doi-asserted-by":"crossref","first-page":"232","DOI":"10.1196\/annals.1293.023","volume":"1011","author":"Dimauro S.","year":"2004","journal-title":"Ann. N.Y. Acad. Sci."},{"key":"p_19\/p_19_1","doi-asserted-by":"crossref","first-page":"217","DOI":"10.1196\/annals.1293.022","volume":"1011","author":"Dimauro S.","year":"2004","journal-title":"Ann. N.Y. Acad. Sci."},{"key":"p_20\/p_20_1","doi-asserted-by":"crossref","first-page":"265","DOI":"10.1023\/B:MEBR.0000020188.57786.0e","volume":"18","author":"Finsterer J.","year":"2003","journal-title":"Metab. Brain Dis."},{"key":"p_21\/p_21_1","first-page":"77","volume":"24","author":"Fischer S.","year":"2005","journal-title":"Clin. Neuropathol."},{"key":"p_22\/p_22_1","doi-asserted-by":"crossref","first-page":"545","DOI":"10.1126\/science.644316","volume":"200","author":"Fishbein W.N.","year":"1978","journal-title":"Science"},{"key":"p_23\/p_23_1","doi-asserted-by":"crossref","first-page":"1835","DOI":"10.1093\/hmg\/5.11.1835","volume":"5","author":"Fu K.","year":"1996","journal-title":"Hum. Mol. Genet."},{"key":"p_24\/p_24_1","doi-asserted-by":"crossref","first-page":"533","DOI":"10.1113\/jphysiol.2005.085043","volume":"566","author":"Fueger P.T.","year":"2005","journal-title":"J. Physiol."},{"key":"p_25\/p_25_1","doi-asserted-by":"crossref","first-page":"1395","DOI":"10.1001\/archneur.59.9.1395","volume":"59","author":"Haller R.G.","year":"2002","journal-title":"Arch. Neurol."},{"key":"p_26\/p_26_1","doi-asserted-by":"crossref","first-page":"82","DOI":"10.1212\/WNL.62.1.82","volume":"62","author":"Haller R.G.","year":"2004","journal-title":"Neurology"},{"key":"p_27\/p_27_1","doi-asserted-by":"crossref","first-page":"217","DOI":"10.1016\/0002-9343(83)90614-9","volume":"74","author":"Haller R.G.","year":"1983","journal-title":"Am. J. Med."},{"key":"p_28\/p_28_1","first-page":"485","volume":"8","author":"Hanisch F.","year":"2003","journal-title":"Eur. J. Med. Res."},{"key":"p_29\/p_29_1","doi-asserted-by":"crossref","first-page":"610","DOI":"10.1016\/j.metabol.2004.12.004","volume":"54","author":"Jentjens R.L.","year":"2005","journal-title":"Metabolism"},{"key":"p_30\/p_30_1","doi-asserted-by":"crossref","first-page":"397","DOI":"10.1093\/ajcn\/67.3.397","volume":"67","author":"Jeukendrup A.E.","year":"1998","journal-title":"Am. J. Clin. Nutr."},{"key":"p_31\/p_31_1","doi-asserted-by":"crossref","first-page":"153","DOI":"10.1002\/ana.10263","volume":"52","author":"Kazemi-Esfarjani P.","year":"2002","journal-title":"Ann. Neurol."},{"key":"p_32\/p_32_1","doi-asserted-by":"crossref","first-page":"1748","DOI":"10.1212\/WNL.55.11.1748","volume":"55","author":"Klopstock T.","year":"2000","journal-title":"Neurology"},{"key":"p_33\/p_33_1","doi-asserted-by":"crossref","first-page":"53","DOI":"10.1016\/S0887-8994(02)00469-1","volume":"28","author":"Komura K.","year":"2003","journal-title":"Pediatr. Neurol."},{"key":"p_34\/p_34_1","doi-asserted-by":"crossref","first-page":"300","DOI":"10.1111\/j.1468-1331.2004.00970.x","volume":"12","author":"Kornblum C.","year":"2005","journal-title":"Eur. J. Neurol."},{"key":"p_35\/p_35_1","doi-asserted-by":"crossref","first-page":"15592","DOI":"10.1073\/pnas.95.26.15592","volume":"95","author":"Kurtz D.M.","year":"1998","journal-title":"Proc. Natl. Acad. Sci. U.S.A."},{"key":"p_36\/p_36_1","doi-asserted-by":"crossref","first-page":"1749","DOI":"10.1152\/jappl.1984.57.6.1749","volume":"57","author":"Lewis S.F.","year":"1984","journal-title":"J. Appl. Physiol."},{"key":"p_37\/p_37_1","doi-asserted-by":"crossref","first-page":"H163","DOI":"10.1152\/ajpheart.01117.2002","volume":"285","author":"Lloyd S.","year":"2003","journal-title":"Am. J. Physiol. Heart Circ. Physiol."},{"key":"p_38\/p_38_1","doi-asserted-by":"crossref","first-page":"435","DOI":"10.1002\/ana.410290416","volume":"29","author":"Matthews P.M.","year":"1991","journal-title":"Ann. Neurol."},{"key":"p_39\/p_39_1","doi-asserted-by":"crossref","first-page":"E580","DOI":"10.1152\/ajpendo.2000.278.4.E580","volume":"278","author":"McKenzie S.","year":"2000","journal-title":"Am. J. Physiol. Endocrinol. Metab."},{"key":"p_40\/p_40_1","doi-asserted-by":"crossref","first-page":"453","DOI":"10.1016\/j.braindev.2003.12.009","volume":"26","author":"Mori M.","year":"2004","journal-title":"Brain Dev."},{"key":"p_41\/p_41_1","doi-asserted-by":"crossref","first-page":"273","DOI":"10.1113\/jphysiol.2005.086025","volume":"566","author":"Mortensen S.P.","year":"2005","journal-title":"J. Physiol."},{"key":"p_42\/p_42_1","doi-asserted-by":"crossref","first-page":"258","DOI":"10.1152\/jappl.2001.91.1.258","volume":"91","author":"Norman B.","year":"2001","journal-title":"J. Appl. Physiol."},{"key":"p_43\/p_43_1","doi-asserted-by":"crossref","first-page":"559","DOI":"10.1212\/01.WNL.0000078195.05396.20","volume":"61","author":"Orngreen M.C.","year":"2003","journal-title":"Neurology"},{"key":"p_44\/p_44_1","doi-asserted-by":"crossref","first-page":"60","DOI":"10.1002\/ana.20320","volume":"57","author":"Orngreen M.C.","year":"2005","journal-title":"Ann. Neurol."},{"key":"p_45\/p_45_1","doi-asserted-by":"crossref","first-page":"27753","DOI":"10.1016\/S0021-9258(19)65048-9","volume":"275","author":"Pederson B.A.","year":"2000","journal-title":"J. Biol. Chem."},{"key":"p_46\/p_46_1","doi-asserted-by":"crossref","first-page":"210","DOI":"10.1016\/S0960-8966(98)00004-2","volume":"8","author":"Phoenix J.","year":"1998","journal-title":"Neuromuscul. Disord."},{"key":"p_47\/p_47_1","doi-asserted-by":"crossref","first-page":"745","DOI":"10.1152\/jappl.1993.75.2.745","volume":"75","author":"Riley M.","year":"1993","journal-title":"J. Appl. Physiol."},{"key":"p_48\/p_48_1","doi-asserted-by":"crossref","first-page":"1419","DOI":"10.1172\/JCI109995","volume":"66","author":"Sabina R.L.","year":"1980","journal-title":"J. Clin. Invest."},{"key":"p_49\/p_49_1","doi-asserted-by":"crossref","first-page":"720","DOI":"10.1172\/JCI111265","volume":"73","author":"Sabina R.L.","year":"1984","journal-title":"J. Clin. Invest."},{"key":"p_50\/p_50_1","doi-asserted-by":"crossref","first-page":"2239","DOI":"10.1093\/hmg\/6.13.2239","volume":"6","author":"Shoubridge E.A.","year":"1997","journal-title":"Hum. Mol. Genet."},{"key":"p_51\/p_51_1","doi-asserted-by":"crossref","first-page":"1541","DOI":"10.1001\/archneur.59.10.1541","volume":"59","author":"Shults C.W.","year":"2002","journal-title":"Arch. Neurol."},{"key":"p_52\/p_52_1","doi-asserted-by":"crossref","first-page":"475","DOI":"10.1042\/cs0720475","volume":"72","author":"Sinkeler S.P.","year":"1987","journal-title":"Clin. Sci. (London)"},{"key":"p_53\/p_53_1","doi-asserted-by":"crossref","first-page":"1422","DOI":"10.1172\/JCI111553","volume":"74","author":"Swain J.L.","year":"1984","journal-title":"J. Clin. Invest."},{"key":"p_54\/p_54_1","doi-asserted-by":"crossref","first-page":"529","DOI":"10.1212\/WNL.47.2.529","volume":"47","author":"Taivassalo T.","year":"1996","journal-title":"Neurology"},{"key":"p_55\/p_55_1","doi-asserted-by":"crossref","first-page":"1055","DOI":"10.1212\/WNL.50.4.1055","volume":"50","author":"Taivassalo T.","year":"1998","journal-title":"Neurology"},{"key":"p_56\/p_56_1","doi-asserted-by":"crossref","first-page":"1047","DOI":"10.1093\/hmg\/8.6.1047","volume":"8","author":"Taivassalo T.","year":"1999","journal-title":"Hum. Mol. Genet."},{"key":"p_57\/p_57_1","doi-asserted-by":"crossref","first-page":"133","DOI":"10.1002\/ana.1050","volume":"50","author":"Taivassalo T.","year":"2001","journal-title":"Ann. Neurol."},{"key":"p_58\/p_58_1","doi-asserted-by":"crossref","first-page":"38","DOI":"10.1002\/ana.10027","volume":"51","author":"Taivassalo T.","year":"2002","journal-title":"Ann. Neurol."},{"key":"p_59\/p_59_1","doi-asserted-by":"crossref","first-page":"413","DOI":"10.1093\/brain\/awg028","volume":"126","author":"Taivassalo T.","year":"2003","journal-title":"Brain"},{"key":"p_60\/p_60_1","doi-asserted-by":"crossref","first-page":"675","DOI":"10.1016\/S0387-7604(02)00074-8","volume":"24","author":"Tamaoki Y.","year":"2002","journal-title":"Brain Dev."},{"key":"p_61\/p_61_1","doi-asserted-by":"crossref","first-page":"1719","DOI":"10.1152\/jappl.2000.89.5.1719","volume":"89","author":"Tarnopolsky M.A.","year":"2000","journal-title":"J. Appl. Physiol."},{"key":"p_62\/p_62_1","doi-asserted-by":"crossref","first-page":"1228","DOI":"10.1002\/(SICI)1097-4598(199909)22:9<1228::AID-MUS9>3.0.CO;2-6","volume":"22","author":"Tarnopolsky M.A.","year":"1999","journal-title":"Muscle Nerve"},{"key":"p_63\/p_63_1","doi-asserted-by":"crossref","first-page":"1502","DOI":"10.1002\/(SICI)1097-4598(199712)20:12<1502::AID-MUS4>3.0.CO;2-C","volume":"20","author":"Tarnopolsky M.A.","year":"1997","journal-title":"Muscle Nerve"},{"key":"p_64\/p_64_1","doi-asserted-by":"crossref","first-page":"881","DOI":"10.1111\/j.1469-7793.2001.t01-1-00881.x","volume":"533","author":"Tarnopolsky M.A.","year":"2001","journal-title":"J. Physiol."},{"key":"p_65\/p_65_1","doi-asserted-by":"crossref","first-page":"359","DOI":"10.1002\/mus.10330","volume":"27","author":"Tarnopolsky M.A.","year":"2003","journal-title":"Muscle Nerve"},{"key":"p_66\/p_66_1","doi-asserted-by":"crossref","first-page":"537","DOI":"10.1002\/mus.20020","volume":"29","author":"Tarnopolsky M.A.","year":"2004","journal-title":"Muscle Nerve"},{"key":"p_67\/p_67_1","doi-asserted-by":"crossref","first-page":"125","DOI":"10.1016\/S0146-0005(99)80046-9","volume":"23","author":"Tein I.","year":"1999","journal-title":"Semin. Perinatol."},{"key":"p_68\/p_68_1","doi-asserted-by":"crossref","first-page":"121","DOI":"10.1016\/j.bbabio.2004.08.006","volume":"1659","author":"Thorburn D.R.","year":"2004","journal-title":"Biochim. Biophys. Acta"},{"key":"p_69\/p_69_1","doi-asserted-by":"crossref","first-page":"417","DOI":"10.1038\/nature02517","volume":"429","author":"Trifunovic A.","year":"2004","journal-title":"Nature"},{"key":"p_70\/p_70_1","doi-asserted-by":"crossref","first-page":"C1067","DOI":"10.1152\/ajpcell.1996.270.4.C1067","volume":"270","author":"Tullson P.C.","year":"1996","journal-title":"Am. J. Physiol."},{"key":"p_71\/p_71_1","doi-asserted-by":"crossref","first-page":"607","DOI":"10.1002\/mus.880130708","volume":"13","author":"Turk W.R.","year":"1990","journal-title":"Muscle Nerve"},{"key":"p_72\/p_72_1","doi-asserted-by":"crossref","first-page":"19921","DOI":"10.1074\/jbc.270.34.19921","volume":"270","author":"Veksler V.I.","year":"1995","journal-title":"J. Biol. Chem."},{"key":"p_73\/p_73_1","doi-asserted-by":"crossref","first-page":"140","DOI":"10.1002\/ana.410440124","volume":"44","author":"Verzijl H.T.","year":"1998","journal-title":"Ann. Neurol."},{"key":"p_74\/p_74_1","doi-asserted-by":"crossref","first-page":"2503","DOI":"10.1056\/NEJMoa031836","volume":"349","author":"Vissing J.","year":"2003","journal-title":"N. Engl. J. Med."},{"key":"p_75\/p_75_1","doi-asserted-by":"crossref","first-page":"655","DOI":"10.1002\/ana.410400416","volume":"40","author":"Vissing J.","year":"1996","journal-title":"Ann. Neurol."},{"key":"p_76\/p_76_1","doi-asserted-by":"crossref","first-page":"956","DOI":"10.1001\/archneur.57.7.956","volume":"57","author":"Vorgerd M.","year":"2000","journal-title":"Arch. Neurol."},{"key":"p_77\/p_77_1","doi-asserted-by":"crossref","first-page":"97","DOI":"10.1001\/archneur.59.1.97","volume":"59","author":"Vorgerd M.","year":"2002","journal-title":"Arch. Neurol."},{"key":"p_78\/p_78_1","doi-asserted-by":"crossref","first-page":"533","DOI":"10.1677\/jme.1.01499","volume":"33","author":"Watt M.J.","year":"2004","journal-title":"J. Mol. Endocrinol."},{"key":"p_79\/p_79_1","doi-asserted-by":"crossref","first-page":"41","DOI":"10.1016\/j.jns.2004.02.003","volume":"220","author":"Winkler-Stuck K.","year":"2004","journal-title":"J. Neurol. Sci."},{"key":"p_80\/p_80_1","doi-asserted-by":"crossref","first-page":"1402","DOI":"10.1212\/01.WNL.0000031795.91814.D8","volume":"59","author":"Wolf N.I.","year":"2002","journal-title":"Neurology"},{"key":"p_81\/p_81_1","doi-asserted-by":"crossref","first-page":"C215","DOI":"10.1152\/ajpcell.00034.2002","volume":"285","author":"Xia R.","year":"2003","journal-title":"Am. J. Physiol. Cell Physiol."},{"key":"p_82\/p_82_1","doi-asserted-by":"crossref","first-page":"728","DOI":"10.1002\/mus.10377","volume":"27","author":"Zange J.","year":"2003","journal-title":"Muscle Nerve"}],"container-title":["Applied Physiology, Nutrition, and Metabolism"],"original-title":[],"language":"en","link":[{"URL":"http:\/\/www.nrcresearchpress.com\/doi\/pdf\/10.1139\/h05-008","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2021,7,24]],"date-time":"2021-07-24T15:19:41Z","timestamp":1627139981000},"score":1,"resource":{"primary":{"URL":"http:\/\/www.nrcresearchpress.com\/doi\/10.1139\/h05-008"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2006,2,1]]},"references-count":82,"journal-issue":{"issue":"1","published-print":{"date-parts":[[2006,2,1]]}},"alternative-id":["10.1139\/h05-008"],"URL":"https:\/\/doi.org\/10.1139\/h05-008","relation":{},"ISSN":["1715-5312","1715-5320"],"issn-type":[{"value":"1715-5312","type":"print"},{"value":"1715-5320","type":"electronic"}],"subject":[],"published":{"date-parts":[[2006,2,1]]}}}