{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2024,6,17]],"date-time":"2024-06-17T20:29:14Z","timestamp":1718656154946},"reference-count":27,"publisher":"Wiley","issue":"7","license":[{"start":{"date-parts":[[2004,10,13]],"date-time":"2004-10-13T00:00:00Z","timestamp":1097625600000},"content-version":"vor","delay-in-days":5218,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Muscle and Nerve"],"published-print":{"date-parts":[[1990,7]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>Enzymes of energy production were measured in muscle homogenates and in individual muscle fibers from 5 patients with McArdle's disease. Individual fibers were investigated to determine whether fibers of all types were completely devoid of glycogen phosphorylase activity and whether the involved fibers might be biochemically altered in a fiber type dependent manner to enhance the energy\u2010generating capabilities of the cells through other metabolic pathways. Using highly sensitive biochemical assays, a complete absence of glycogen phosphorylase, a and b, activity was found in fibers of all types in the McArdle's patients. Levels of enzymes representing glycolysis, the Krebs cycle, and high energy phosphate metabolism were essentially normal in each fiber type, indicating an apparent lack in metabolic adaptation of these energy pathways to the absence of fatty acids (\u03b2\u2010hydrosyacyl CoA dehydrogenase, \u03b2OAC) was elevated in all patients, and substantially in 4 of the 5. This suggested that lipid substrates can provide support for oxidative endurance capacity in some patients. Individual fiber analysises indicated that the compensation involved fibers of all types.<\/jats:p>","DOI":"10.1002\/mus.880130708","type":"journal-article","created":{"date-parts":[[2005,1,1]],"date-time":"2005-01-01T09:40:50Z","timestamp":1104572450000},"page":"607-612","source":"Crossref","is-referenced-by-count":6,"title":["Increased muscular \u03b2\u2010hydroxyacyl CoA dehydrogenase with McArdle's disease"],"prefix":"10.1002","volume":"13","author":[{"given":"William R.","family":"Turk","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Scott L.","family":"Heller","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Beverly J.","family":"Norris","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Patti M.","family":"Nemeth","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2004,10,13]]},"reference":[{"key":"e_1_2_1_2_2","article-title":"Effects of detraining on enzymes of energy metabolism in individual human muscle fibres","volume":"244","author":"Chi MM\u2010Y","year":"1983","journal-title":"Am J Physiol"},{"key":"e_1_2_1_3_2","article-title":"Chronic stimulation of mammalian muscle: Enzyme changes in individual fibers","volume":"251","author":"Chi MM\u2010Y","year":"1986","journal-title":"Am J Physiol"},{"key":"e_1_2_1_4_2","doi-asserted-by":"publisher","DOI":"10.1016\/0026-0495(87)90113-2"},{"key":"e_1_2_1_5_2","doi-asserted-by":"publisher","DOI":"10.1016\/0003-2697(78)90732-7"},{"key":"e_1_2_1_6_2","doi-asserted-by":"publisher","DOI":"10.1212\/WNL.28.11.1124"},{"key":"e_1_2_1_7_2","doi-asserted-by":"crossref","first-page":"60","DOI":"10.1159\/000459414","article-title":"Molecular studies on glycogen storage disease","volume":"18","author":"Dreyus J\u2010C","year":"1974","journal-title":"Enzyme"},{"key":"e_1_2_1_8_2","doi-asserted-by":"publisher","DOI":"10.1056\/NEJM196301172680306"},{"key":"e_1_2_1_9_2","doi-asserted-by":"publisher","DOI":"10.1212\/WNL.26.10.963"},{"key":"e_1_2_1_10_2","doi-asserted-by":"publisher","DOI":"10.1212\/WNL.37.6.1039"},{"key":"e_1_2_1_11_2","doi-asserted-by":"publisher","DOI":"10.1177\/32.6.6202737"},{"key":"e_1_2_1_12_2","doi-asserted-by":"crossref","first-page":"111","DOI":"10.1016\/0022-510X(73)90023-3","article-title":"Data on the distribution of fiber types in thirty\u2010six human muscles; an autopsy study","volume":"18","author":"Johnson MA","journal-title":"J Neurol Sci"},{"key":"e_1_2_1_13_2","doi-asserted-by":"crossref","first-page":"203","DOI":"10.1152\/jappl.1971.31.2.203","article-title":"Diet, muscle glycogen and endurance performance","volume":"31","author":"Karlson J","year":"1971","journal-title":"J Appl Physiol"},{"key":"e_1_2_1_14_2","doi-asserted-by":"publisher","DOI":"10.1016\/0003-2697(73)90409-0"},{"key":"e_1_2_1_15_2","doi-asserted-by":"publisher","DOI":"10.1002\/mus.880030302"},{"key":"e_1_2_1_16_2","doi-asserted-by":"crossref","first-page":"8269","DOI":"10.1016\/S0021-9258(17)34391-0","article-title":"Enzyme pattern in single human muscle fibres","volume":"253","author":"Lowry CV","year":"1978","journal-title":"J Biol Chem"},{"key":"e_1_2_1_17_2","volume-title":"A Flexible System of Enzymatic Analysis","author":"Lowry OH","year":"1972"},{"key":"e_1_2_1_18_2","first-page":"13","article-title":"Myopathy due to a defect in muscle glycogen breakdown","volume":"10","author":"McArdle B","year":"1951","journal-title":"Clin Sci"},{"key":"e_1_2_1_19_2","first-page":"78","article-title":"Adaptations to a highfat diet that increase exercise endurance in male rats","volume":"56","author":"Miller WC","year":"1984","journal-title":"J Appl Physiol Respirat Environ Exercise Physiol"},{"key":"e_1_2_1_20_2","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.45.6.791"},{"key":"e_1_2_1_21_2","first-page":"258","volume-title":"Segmental Motor Systems","author":"Nemeth PM","year":"1990"},{"key":"e_1_2_1_22_2","doi-asserted-by":"publisher","DOI":"10.1177\/32.11.6491255"},{"key":"e_1_2_1_23_2","doi-asserted-by":"publisher","DOI":"10.1523\/JNEUROSCI.06-03-00892.1986"},{"key":"e_1_2_1_24_2","doi-asserted-by":"publisher","DOI":"10.1007\/BF01467777"},{"key":"e_1_2_1_25_2","doi-asserted-by":"publisher","DOI":"10.1021\/bi00764a013"},{"key":"e_1_2_1_26_2","doi-asserted-by":"publisher","DOI":"10.1172\/JCI103983"},{"key":"e_1_2_1_27_2","doi-asserted-by":"publisher","DOI":"10.1056\/NEJM198502073120607"},{"key":"e_1_2_1_28_2","doi-asserted-by":"publisher","DOI":"10.1097\/00000441-197503000-00008"}],"container-title":["Muscle &amp; 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