{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,9]],"date-time":"2026-10-09T19:11:17Z","timestamp":1791573077475,"version":"4.3.4"},"reference-count":47,"publisher":"National Academy of Sciences","issue":"8","content-domain":{"domain":["www.pnas.org"],"crossmark-restriction":true},"short-container-title":["Proc. Natl. Acad. Sci. U.S.A."],"published-print":{"date-parts":[[2007,2,20]]},"abstract":"<jats:p>\n                    Activity of complexes II, III, and IV of the mitochondrial electron transport system (ETS) is reduced in postmortem Huntington's disease (HD) striatum, suggesting that reduced cerebral oxidative phosphorylation may be important in the pathogenesis of neuronal death. We investigated mitochondrial oxidative metabolism\n                    <jats:italic>in vivo<\/jats:italic>\n                    in the striatum of 20 participants with early, genetically proven HD and 15 age-matched normal controls by direct measurements of the molar ratio of cerebral oxygen metabolism to cerebral glucose metabolism (CMRO\n                    <jats:sub>2<\/jats:sub>\n                    \/CMRglc) with positron emission tomography. There was a significant increase in striatal CMRO\n                    <jats:sub>2<\/jats:sub>\n                    \/CMRglc in HD rather than the decrease characteristic of defects in mitochondrial oxidative metabolism (6.0 \u00b1 1.6 vs. 5.1 \u00b1 0.9,\n                    <jats:italic>P<\/jats:italic>\n                    = 0.04). CMRO\n                    <jats:sub>2<\/jats:sub>\n                    was not different from controls (126 \u00b1 37 vs. 134 \u00b1 31 \u03bcmol 100 g\n                    <jats:sup>\u22121<\/jats:sup>\n                    min\n                    <jats:sup>\u22121<\/jats:sup>\n                    ,\n                    <jats:italic>P<\/jats:italic>\n                    = 0.49), whereas CMRglc was decreased (21.6 \u00b1 6.1 vs. 26.4 \u00b1 4.6 \u03bcmol 100 g\n                    <jats:sup>\u22121<\/jats:sup>\n                    min\n                    <jats:sup>\u22121<\/jats:sup>\n                    ,\n                    <jats:italic>P<\/jats:italic>\n                    = 0.01). Striatal volume was decreased as well (13.9 \u00b1 3.5 vs. 17.6 \u00b1 2.0 ml,\n                    <jats:italic>P<\/jats:italic>\n                    = 0.001). Increased striatal CMRO\n                    <jats:sub>2<\/jats:sub>\n                    \/CMRglc with unchanged CMRO\n                    <jats:sub>2<\/jats:sub>\n                    is inconsistent with a defect in mitochondrial oxidative phosphorylation due to reduced activity of the mitochondrial ETS. Because HD pathology was already manifest by striatal atrophy, deficient energy production due to a reduced activity of the mitochondrial ETS is not important in the mechanism of neuronal death in early HD. Because glycolytic metabolism is predominantly astrocytic, the selective reduction in striatal CMRglc raises the possibility that astrocyte dysfunction may be involved in the pathogenesis of HD.\n                  <\/jats:p>","DOI":"10.1073\/pnas.0609833104","type":"journal-article","created":{"date-parts":[[2007,2,13]],"date-time":"2007-02-13T21:48:54Z","timestamp":1171403334000},"page":"2945-2949","update-policy":"https:\/\/doi.org\/10.1073\/pnas.cm10313","source":"Crossref","is-referenced-by-count":158,"title":["Selective defect of\n                    <i>in vivo<\/i>\n                    glycolysis in early Huntington's disease striatum"],"prefix":"10.1073","volume":"104","author":[{"given":"William J.","family":"Powers","sequence":"first","affiliation":[{"name":"Departments of *Neurology,"},{"name":"Neurological Surgery,"},{"name":"Mallinckrodt Institute of Radiology, Washington University School of Medicine, Campus Box 8225, 4525 Scott Avenue, St. Louis, MO 63110"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Tom O.","family":"Videen","sequence":"additional","affiliation":[{"name":"Departments of *Neurology,"},{"name":"Mallinckrodt Institute of Radiology, Washington University School of Medicine, Campus Box 8225, 4525 Scott Avenue, St. Louis, MO 63110"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Joanne","family":"Markham","sequence":"additional","affiliation":[{"name":"Mallinckrodt Institute of Radiology, Washington University School of Medicine, Campus Box 8225, 4525 Scott Avenue, St. Louis, MO 63110"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lori","family":"McGee-Minnich","sequence":"additional","affiliation":[{"name":"Departments of *Neurology,"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"JoAnn V.","family":"Antenor-Dorsey","sequence":"additional","affiliation":[{"name":"Departments of 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