{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,3,14]],"date-time":"2026-03-14T03:50:44Z","timestamp":1773460244637,"version":"3.50.1"},"reference-count":20,"publisher":"Wiley","issue":"2","license":[{"start":{"date-parts":[[2004,10,7]],"date-time":"2004-10-07T00:00:00Z","timestamp":1097107200000},"content-version":"vor","delay-in-days":7188,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Annals of Neurology"],"published-print":{"date-parts":[[1985,2]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>We studied oxidative metabolism during bicycle exercise in 4 patients with myophosphorylase deficiency. Maximal oxygen uptake (VO<jats:sub>2<\/jats:sub>max) was low (14.0 \u00b1 1.4ml\u00b7kg<jats:sup>\u22121<\/jats:sup>\u00b7 min<jats:sup>\u22121<\/jats:sup>, mean \u00b1 SE) compared with that in normal subjects (37.7 \u00b1 1.9; n = 12) and patients with myalgia (24.9 \u00b1 1.8; n = 10). Carbohydrate oxidation, as estimated by the respiratory exchange ratio (R), was low relative to workload (max R, mean \u00b1 SE: McArdle's disease, 0.96 \u00b1 0.02; normal subject, 1.13 \u00b1 0.02; myalgia, 1.09 \u00b1 0.02). Intravenous glucose administration increased maximal oxygen uptake about 20% in those with McArdle's disease, but both VO<jats:sub>2<\/jats:sub>max and R remained lower than in control subjects. These findings suggest that the capacity for dynamic exercise in McArdle's disease is limited by the availability of oxidative substrate, and indicate that blood glucose is unable to substitute fully for muscle glycogen as an oxidative fuel. We also found that exercise cardiac output (Q) was excessive relative to oxygen uptake in affected patients (\u03b4Q\u0307\/\u03b4V\u0307O<jats:sub>2<\/jats:sub>, mean \u00b1 SE: McArdle's disease, 11.6 \u00b1 1.7; normal subjects, 48 \u00b1 0.2; myalgia, 5.6 \u00b1 0.2). This hyperkinetic circulation in exercise may serve to increase the delivery of blood\u2010brone oxidative substrate to working muscle.<\/jats:p>","DOI":"10.1002\/ana.410170216","type":"journal-article","created":{"date-parts":[[2005,1,1]],"date-time":"2005-01-01T17:19:42Z","timestamp":1104599982000},"page":"196-199","source":"Crossref","is-referenced-by-count":51,"title":["Myophosphorylase deficiency impairs muscle oxidative metabolism"],"prefix":"10.1002","volume":"17","author":[{"given":"Ronald G.","family":"Haller","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Steven F.","family":"Lewis","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"J. D.","family":"Cook","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"C. G.","family":"Blomqvist","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2004,10,7]]},"reference":[{"key":"e_1_2_1_2_2","volume-title":"Textbook of Work Physiology: Physiological Basis of Exercise","author":"Astrand P\u2010O","year":"1977"},{"key":"e_1_2_1_3_2","first-page":"44p","article-title":"The effect of exercise on forearm blood flow and on venous blood pH, PCO2 and lactate in a subject with phosphorylase deficiency in skilital muscle (McArdle's syndrome)","volume":"189","author":"Barcroft H","year":"1966","journal-title":"J Physiol (Lond)"},{"key":"e_1_2_1_4_2","doi-asserted-by":"publisher","DOI":"10.1146\/annurev.ph.45.030183.001125"},{"key":"e_1_2_1_5_2","doi-asserted-by":"publisher","DOI":"10.1001\/archneur.1979.00500440027003"},{"key":"e_1_2_1_6_2","doi-asserted-by":"publisher","DOI":"10.1113\/jphysiol.1977.sp011726"},{"key":"e_1_2_1_7_2","first-page":"148","article-title":"The cardiac output\u2013oxygen uptake relationship of men during graded bicycle ergometry","volume":"9","author":"Faulkner JA","year":"1977","journal-title":"Med Sci Sports"},{"key":"e_1_2_1_8_2","doi-asserted-by":"publisher","DOI":"10.1056\/NEJM197511202932107"},{"key":"e_1_2_1_9_2","doi-asserted-by":"crossref","first-page":"991","DOI":"10.1152\/jappl.1982.52.4.991","article-title":"Exercise hyperventilation in McArdle's disease","volume":"52","author":"Hagberg JM","year":"1982","journal-title":"J Appl Phsiol"},{"key":"e_1_2_1_10_2","doi-asserted-by":"publisher","DOI":"10.1212\/WNL.33.10.1283"},{"key":"e_1_2_1_11_2","doi-asserted-by":"publisher","DOI":"10.3109\/00365516909080140"},{"key":"e_1_2_1_12_2","doi-asserted-by":"publisher","DOI":"10.1136\/jnnp.27.5.361"},{"key":"e_1_2_1_13_2","unstructured":"LewisSF HallerRG CookJD BlomqvistCG: Metabolic control of the cardiac output response to exercise in McArdle's disease.J Appl Physiol(in press)"},{"key":"e_1_2_1_14_2","doi-asserted-by":"publisher","DOI":"10.1111\/j.0954-6820.1969.tb07314.x"},{"key":"e_1_2_1_15_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_16_2","doi-asserted-by":"publisher","DOI":"10.1016\/0002-9343(61)90075-4"},{"key":"e_1_2_1_17_2","doi-asserted-by":"crossref","first-page":"294","DOI":"10.1111\/j.0954-6820.1967.tb12635.x","article-title":"The second wind phenomenon in McArdle's syndrome","volume":"472","author":"Pernow BB","year":"1967","journal-title":"Acta Med Scand"},{"key":"e_1_2_1_18_2","doi-asserted-by":"publisher","DOI":"10.1056\/NEJM196608252750802"},{"key":"e_1_2_1_19_2","first-page":"1","volume-title":"Carbohydrate Metabolism and Its Disorders","author":"Rennie MJ","year":"1981"},{"key":"e_1_2_1_20_2","first-page":"185","volume-title":"The Structure and Function of Muscle","author":"Rosell S","year":"1973"},{"key":"e_1_2_1_21_2","first-page":"203","article-title":"Non\u2010invasive determination of cardiac output by a modified acetylende rebreathing procedure using mass spectrometer measurements","volume":"48","author":"Triebwasser JH","year":"1977","journal-title":"Aviat Space Enviorn Med"}],"container-title":["Annals of Neurology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1002%2Fana.410170216","content-type":"unspecified","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/ana.410170216","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2024,12,22]],"date-time":"2024-12-22T05:45:35Z","timestamp":1734846335000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1002\/ana.410170216"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[1985,2]]},"references-count":20,"journal-issue":{"issue":"2","published-print":{"date-parts":[[1985,2]]}},"alternative-id":["10.1002\/ana.410170216"],"URL":"https:\/\/doi.org\/10.1002\/ana.410170216","archive":["Portico"],"relation":{},"ISSN":["0364-5134","1531-8249"],"issn-type":[{"value":"0364-5134","type":"print"},{"value":"1531-8249","type":"electronic"}],"subject":[],"published":{"date-parts":[[1985,2]]}}}