{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,5]],"date-time":"2026-10-05T11:09:20Z","timestamp":1791198560540,"version":"4.1.0"},"reference-count":30,"publisher":"Wiley","issue":"12","license":[{"start":{"date-parts":[[2007,6,7]],"date-time":"2007-06-07T00:00:00Z","timestamp":1181174400000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Movement Disorders"],"published-print":{"date-parts":[[2007,9,15]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:p>\n                    Huntington's disease (HD) is caused by an abnormally expanded CAG repeat in the\n                    <jats:italic>IT<\/jats:italic>\n                    \u2010\n                    <jats:italic>15<\/jats:italic>\n                    gene, which encodes a widely expressed protein called huntingtin. Abnormalities of mitochondrial respiratory chain function, specifically complex II\/III, have been identified in HD striatum and defects of energy metabolism have been demonstrated in vivo in skeletal muscle in both symptomatic and presymptomatic HD patients. We have investigated respiratory chain function using histochemical and biochemical methods in HD skeletal muscle from 12 patients and compared these with 12 age and sex\u2010matched controls. The data from the HD patients were related to clinical parameters of HD including the Unified Huntington's Disease Rating Scale (UHDRS). There were positive correlations between CAG repeat years (a product of CAG repeat length and age) and both motor (\n                    <jats:italic>P<\/jats:italic>\n                    &lt; 0.002) and cognitive (\n                    <jats:italic>P<\/jats:italic>\n                    &lt; 0.01) scores of the UHDRS. There was no significant difference in the activities of complexes I to IV compared to age\u2010matched controls. However, there were significant correlations for individual HD complex II\/III activities with disease duration (\n                    <jats:italic>P<\/jats:italic>\n                    = 0.017), repeat years (\n                    <jats:italic>P<\/jats:italic>\n                    = 0.032), and cognitive scores (\n                    <jats:italic>P<\/jats:italic>\n                    = 0.019). There was also evidence from ultrastructural studies that inclusion formation may occur in HD muscle. These results provide additional evidence that mutant huntingtin influences mitochondrial complex II\/III function in non\u2010neuronal tissue (skeletal muscle) and suggest that muscle may be a potential marker of disease progression in HD. \u00a9 2007 Movement Disorder Society\n                  <\/jats:p>","DOI":"10.1002\/mds.21540","type":"journal-article","created":{"date-parts":[[2007,6,7]],"date-time":"2007-06-07T16:56:25Z","timestamp":1181235385000},"page":"1715-1721","source":"Crossref","is-referenced-by-count":89,"title":["Clinical correlates of mitochondrial function in Huntington's disease muscle"],"prefix":"10.1002","volume":"22","author":[{"given":"Christopher","family":"Turner","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"J. Mark","family":"Cooper","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Anthony H. V.","family":"Schapira","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2007,6,7]]},"reference":[{"key":"e_1_2_6_2_2","doi-asserted-by":"publisher","DOI":"10.1016\/0092-8674(93)90585-E"},{"key":"e_1_2_6_3_2","doi-asserted-by":"publisher","DOI":"10.1016\/S0092-8674(00)80513-9"},{"key":"e_1_2_6_4_2","doi-asserted-by":"publisher","DOI":"10.1126\/science.277.5334.1990"},{"key":"e_1_2_6_5_2","first-page":"1202","article-title":"The likelihood of being affected with Huntington disease by a particular age, for a specific CAG size","volume":"60","author":"Brinkman RR","year":"1997","journal-title":"Am J Hum Genet"},{"key":"e_1_2_6_6_2","doi-asserted-by":"publisher","DOI":"10.1016\/S0140-6736(03)13304-1"},{"key":"e_1_2_6_7_2","doi-asserted-by":"publisher","DOI":"10.1038\/sj.embor.7400250"},{"key":"e_1_2_6_8_2","doi-asserted-by":"publisher","DOI":"10.1126\/science.1059581"},{"key":"e_1_2_6_9_2","doi-asserted-by":"publisher","DOI":"10.1136\/jnnp.2003.017822"},{"key":"e_1_2_6_10_2","doi-asserted-by":"publisher","DOI":"10.1016\/S0005-2728(98)00115-7"},{"key":"e_1_2_6_11_2","doi-asserted-by":"publisher","DOI":"10.1002\/ana.410390317"},{"key":"e_1_2_6_12_2","doi-asserted-by":"publisher","DOI":"10.1002\/ana.410410514"},{"key":"e_1_2_6_13_2","doi-asserted-by":"publisher","DOI":"10.1002\/mds.20373"},{"key":"e_1_2_6_14_2","doi-asserted-by":"publisher","DOI":"10.1002\/1531-8249(200007)48:1<72::AID-ANA11>3.0.CO;2-I"},{"key":"e_1_2_6_15_2","doi-asserted-by":"publisher","DOI":"10.1002\/ana.410430321"},{"key":"e_1_2_6_16_2","doi-asserted-by":"publisher","DOI":"10.1038\/13518"},{"key":"e_1_2_6_17_2","doi-asserted-by":"publisher","DOI":"10.1093\/hmg\/ddi319"},{"key":"e_1_2_6_18_2","doi-asserted-by":"publisher","DOI":"10.1002\/mds.870130609"},{"key":"e_1_2_6_19_2","volume-title":"Theory and practice of histotechnology","author":"Sheehan DC","year":"1987"},{"key":"e_1_2_6_20_2","doi-asserted-by":"publisher","DOI":"10.1111\/j.1471-4159.1990.tb05809.x"},{"key":"e_1_2_6_21_2","doi-asserted-by":"publisher","DOI":"10.1093\/hmg\/9.2.275"},{"key":"e_1_2_6_22_2","doi-asserted-by":"publisher","DOI":"10.1016\/0003-2697(88)90383-1"},{"key":"e_1_2_6_23_2","doi-asserted-by":"publisher","DOI":"10.1212\/WNL.50.5.1357"},{"key":"e_1_2_6_24_2","doi-asserted-by":"publisher","DOI":"10.1046\/j.1471-4159.2003.02009.x"},{"key":"e_1_2_6_25_2","doi-asserted-by":"publisher","DOI":"10.1093\/hmg\/ddi192"},{"key":"e_1_2_6_26_2","doi-asserted-by":"publisher","DOI":"10.1093\/geronj\/47.3.B71"},{"key":"e_1_2_6_27_2","doi-asserted-by":"publisher","DOI":"10.1212\/WNL.57.4.658"},{"key":"e_1_2_6_28_2","doi-asserted-by":"publisher","DOI":"10.1152\/japplphysiol.01102.2006"},{"key":"e_1_2_6_29_2","doi-asserted-by":"publisher","DOI":"10.1002\/1531-8249(199901)45:1<25::AID-ART6>3.0.CO;2-E"},{"key":"e_1_2_6_30_2","doi-asserted-by":"publisher","DOI":"10.1093\/hmg\/ddl248"},{"key":"e_1_2_6_31_2","doi-asserted-by":"publisher","DOI":"10.1212\/WNL.57.3.397"}],"container-title":["Movement Disorders"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1002%2Fmds.21540","content-type":"unspecified","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/movementdisorders.onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/mds.21540","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,10,20]],"date-time":"2025-10-20T12:32:24Z","timestamp":1760963544000},"score":1,"resource":{"primary":{"URL":"https:\/\/movementdisorders.onlinelibrary.wiley.com\/doi\/10.1002\/mds.21540"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2007,6,7]]},"references-count":30,"journal-issue":{"issue":"12","published-print":{"date-parts":[[2007,9,15]]}},"alternative-id":["10.1002\/mds.21540"],"URL":"https:\/\/doi.org\/10.1002\/mds.21540","archive":["Portico"],"relation":{},"ISSN":["0885-3185","1531-8257"],"issn-type":[{"value":"0885-3185","type":"print"},{"value":"1531-8257","type":"electronic"}],"subject":[],"published":{"date-parts":[[2007,6,7]]}}}