{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,6]],"date-time":"2026-10-06T18:10:54Z","timestamp":1791310254309,"version":"4.3.1"},"reference-count":17,"publisher":"S. Karger AG","issue":"4","license":[{"start":{"date-parts":[[2010,12,3]],"date-time":"2010-12-03T00:00:00Z","timestamp":1291334400000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/www.karger.com\/Services\/SiteLicenses"},{"start":{"date-parts":[[2010,12,3]],"date-time":"2010-12-03T00:00:00Z","timestamp":1291334400000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.karger.com\/Services\/SiteLicenses"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Neurodegener Dis"],"published-print":{"date-parts":[[2011]]},"abstract":"<i>Background:<\/i> Huntington\u2019s disease (HD) is associated with impaired energy metabolism in the brain. Creatine kinase (CK) catalyzes ATP-dependent phosphorylation of creatine (Cr) into phosphocreatine (PCr), thereby serving as readily available high-capacity spatial and temporal ATP buffering. <i>Objective:<\/i> Substantial evidence supports a specific role of the Cr\/PCr system in neurodegenerative diseases. In the brain, the Cr\/PCr ATP-buffering system is established by a concerted operation of the brain-specific cytosolic enzyme BB-CK and ubiquitous mitochondrial uMt-CK. It is not yet established whether the activity of these CK isoenzymes is impaired in HD. <i>Methods:<\/i> We measured PCr, Cr, ATP and ADP in brain extracts of 3 mouse models of HD \u2013 R6\/2 mice, N171-82Q and HdhQ<sup>111<\/sup> mice \u2013 and the activity of CK in cytosolic and mitochondrial brain fractions from the same mice. <i>Results:<\/i> The PCr was significantly increased in mouse HD brain extracts as compared to nontransgenic littermates. We also found an approximately 27% decrease in CK activity in both cytosolic and mitochondrial fractions of R6\/2 and N171-82Q mice, and an approximately 25% decrease in the mitochondria from HdhQ<sup>111<\/sup> mice. Moreover, uMt-CK and BB-CK activities were approximately 63% lower in HD human brain samples as compared to nondiseased controls. <i>Conclusion:<\/i> Our findings lend strong support to the role of impaired energy metabolism in HD, and point out the potential importance of impairment of the CK-catalyzed ATP-buffering system in the etiology of HD.<\/jats:p>","DOI":"10.1159\/000321681","type":"journal-article","created":{"date-parts":[[2010,12,3]],"date-time":"2010-12-03T07:49:01Z","timestamp":1291362541000},"page":"194-201","source":"Crossref","is-referenced-by-count":47,"title":["Impaired Brain Creatine Kinase Activity in Huntington\u2019s Disease"],"prefix":"10.1159","volume":"8","author":[{"given":"S.F.","family":"Zhang","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"T.","family":"Hennessey","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"L.","family":"Yang","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"N.N.","family":"Starkova","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"M.F.","family":"Beal","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"A.A.","family":"Starkov","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"127","published-online":{"date-parts":[[2010,12,3]]},"reference":[{"key":"ref1","doi-asserted-by":"publisher","DOI":"10.1155%2FJBB%2F2006%2F35936"},{"key":"ref2","doi-asserted-by":"publisher","DOI":"10.1093%2Fhmg%2F11.17.1911"},{"key":"ref3","doi-asserted-by":"publisher","DOI":"10.1074%2Fmcp.M500090-MCP200"},{"key":"ref4","doi-asserted-by":"publisher","DOI":"10.1111%2Fj.1471-4159.2006.04323.x"},{"key":"ref5","doi-asserted-by":"publisher","DOI":"10.1023%2FB%3ANERE.0000014824.04728.dd"},{"key":"ref6","doi-asserted-by":"publisher","DOI":"10.1111%2Fj.1471-4159.1990.tb04189.x"},{"key":"ref7","doi-asserted-by":"publisher","DOI":"10.3109%2F00365517909104932"},{"key":"ref8","doi-asserted-by":"publisher","DOI":"10.1016%2FS0092-8674%2800%2980186-5"},{"key":"ref9","doi-asserted-by":"publisher","DOI":"10.1111%2Fj.1469-7793.2003.00847.x"},{"key":"ref10","doi-asserted-by":"publisher","DOI":"10.1111%2Fj.1471-4159.2004.02599.x"},{"key":"ref11","doi-asserted-by":"publisher","DOI":"10.1046%2Fj.1471-4159.2001.00052.x"},{"key":"ref12","doi-asserted-by":"publisher","DOI":"10.1016%2Fj.bbr.2004.07.002"},{"key":"ref13","doi-asserted-by":"publisher","DOI":"10.1111%2Fj.1460-9568.2004.03478.x"},{"key":"ref14","doi-asserted-by":"publisher","DOI":"10.1111%2Fj.1471-4159.1992.tb11028.x"},{"key":"ref15","doi-asserted-by":"publisher","DOI":"10.1023%2FA%3A1022409101641"},{"key":"ref16","doi-asserted-by":"publisher","DOI":"10.1073%2Fpnas.110078997"},{"key":"ref17","doi-asserted-by":"publisher","DOI":"10.1089%2Fars.2006.8.2061"}],"container-title":["Neurodegenerative Diseases"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.karger.com\/Article\/Pdf\/321681","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/www.karger.com\/Article\/Pdf\/321681","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,4,23]],"date-time":"2025-04-23T19:02:31Z","timestamp":1745434951000},"score":1,"resource":{"primary":{"URL":"https:\/\/karger.com\/article\/doi\/10.1159\/000321681"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2010,12,3]]},"references-count":17,"journal-issue":{"issue":"4","published-online":{"date-parts":[[2011,5,1]]},"published-print":{"date-parts":[[2011,5,10]]}},"URL":"https:\/\/doi.org\/10.1159\/000321681","archive":["Portico"],"relation":{},"ISSN":["1660-2854","1660-2862"],"issn-type":[{"value":"1660-2854","type":"print"},{"value":"1660-2862","type":"electronic"}],"subject":[],"published":{"date-parts":[[2010,12,3]]}}}