{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,4,8]],"date-time":"2026-04-08T02:57:39Z","timestamp":1775617059990,"version":"3.50.1"},"reference-count":41,"publisher":"Wiley","issue":"5","license":[{"start":{"date-parts":[[2016,2,4]],"date-time":"2016-02-04T00:00:00Z","timestamp":1454544000000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"},{"start":{"date-parts":[[2016,2,4]],"date-time":"2016-02-04T00:00:00Z","timestamp":1454544000000},"content-version":"unspecified","delay-in-days":0,"URL":"http:\/\/www.springer.com\/tdm"}],"content-domain":{"domain":["link.springer.com"],"crossmark-restriction":false},"short-container-title":["Lipids"],"published-print":{"date-parts":[[2016,5]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>Huntington's Disease (HD) is an autosomal dominant disease that occurs as a result of expansion of the trinucleotide repeat CAG (glutamine) on the <jats:italic>HTT<\/jats:italic> gene. HD patients exhibit various forms of mitochondrial dysfunction within neurons and peripheral tissues. Cardiolipin (Ptd<jats:sub>2<\/jats:sub>Gro) is a polyglycerophospholipid found exclusively in mitochondria and is important for maintaining mitochondrial function. We examined if altered Ptd<jats:sub>2<\/jats:sub>Gro metabolism was involved in the mitochondrial dysfunction associated with HD. Mitochondrial basal respiration, spare respiratory capacity, ATP coupling efficiency and rate of glycolysis were markedly diminished in Epstein\u2010Barr virus transformed HD lymphoblasts compared to controls (CTRL). Mitochondrial supercomplex formation and Complex I activity within these supercomplexes did not vary between HD patients with different length of CAG repeats and appeared unaltered compared to CTRL. In contrast, <jats:italic>in vitro<\/jats:italic> Complex I enzyme activity in mitochondrial enriched samples was reduced in HD lymphoblasts compared to CTRL. The total cellular pool size of Ptd<jats:sub>2<\/jats:sub>Gro and its synthesis\/remodeling from [<jats:sup>3<\/jats:sup>H]acetate\/[<jats:sup>14<\/jats:sup>C]oleate were unaltered in HD lymphoblasts compared to CTRL. In addition, the molecular species of Ptd<jats:sub>2<\/jats:sub>Gro were essentially unaltered in HD lymphoblasts compared to CTRL. We conclude that compared to CTRL lymphoblasts, HD lymphoblasts display impaired mitochondrial basal respiration, spare respiratory capacity, ATP coupling efficiency and rate of glycolysis with any pathological CAG repeat length, but this is not due to alterations in Ptd<jats:sub>2<\/jats:sub>Gro metabolism. We suggest that HD patient lymphoblasts may be a useful model to study defective energy metabolism that does not involve alterations in Ptd<jats:sub>2<\/jats:sub>Gro metabolism.<\/jats:p>","DOI":"10.1007\/s11745-015-4110-0","type":"journal-article","created":{"date-parts":[[2016,2,4]],"date-time":"2016-02-04T05:44:37Z","timestamp":1454564677000},"page":"561-569","update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":21,"title":["Reduced Mitochondrial Function in Human Huntington Disease Lymphoblasts is Not Due to Alterations in Cardiolipin Metabolism or Mitochondrial Supercomplex Assembly"],"prefix":"10.1002","volume":"51","author":[{"given":"Edgard M.","family":"Mejia","sequence":"first","affiliation":[{"name":"Department of Pharmacology and Therapeutics, Faculty of Health Sciences University of Manitoba 501C\u2010715 McDermot Avenue Winnipeg MB R3E 3P4 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