{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,7]],"date-time":"2026-10-07T20:30:06Z","timestamp":1791405006274,"version":"4.3.3"},"reference-count":25,"publisher":"S. Karger AG","issue":"6","license":[{"start":{"date-parts":[[2011,1,1]],"date-time":"2011-01-01T00:00:00Z","timestamp":1293840000000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/www.karger.com\/Services\/SiteLicenses"},{"start":{"date-parts":[[2011,1,1]],"date-time":"2011-01-01T00:00:00Z","timestamp":1293840000000},"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":"<jats:p>&lt;i&gt;Background:&lt;\/i&gt; Reduced peroxisome proliferator-activated receptor-\u03b3 coactivator 1\u03b1 (PGC1\u03b1) gene expression has been observed in striatal cell lines, transgenic mouse models of Huntington\u2019s disease (HD), and brain tissue from HD patients. As this protein is a key transcription regulator of the expression of many mitochondrial proteins, these observations strongly support the role of aberrant mitochondrial function in the pathogenesis of HD. The PGC1\u03b1 protein undergoes posttranslational modifications that affect its transcriptional activity. The N-truncated splice variant of PGC1\u03b1 (NT-PGC1\u03b1) is produced in tissues, but the role of truncated splice variants of PGC1\u03b1 in HD and in the regulation of mitochondrial gene expression has not been elucidated. &lt;i&gt;Objective:&lt;\/i&gt; To examine the expression and modulation of expression of NT-PGC1\u03b1 levels in HD. &lt;i&gt;Methods and Results:&lt;\/i&gt; We found that the NT-PGC1\u03b1 protein, a splice variant of \u223c38 kDa, but not full-length PGC1\u03b1 is severely and consistently altered in human HD brain, human HD myoblasts, mouse HD models, and HD striatal cells. NT-PGC1\u03b1 levels were significantly upregulated in HD cells and mouse brown fat by physiologically relevant stimuli that are known to upregulate PGC1\u03b1 gene expression. This resulted in an increase in mitochondrial gene expression and cytochrome c content. &lt;i&gt;Conclusion:&lt;\/i&gt; Our data suggest that NT-PGC1\u03b1 is an important component of the PGC1\u03b1 transcriptional network, which plays a significant role in the pathogenesis of HD.<\/jats:p>","DOI":"10.1159\/000327910","type":"journal-article","created":{"date-parts":[[2011,7,15]],"date-time":"2011-07-15T08:15:26Z","timestamp":1310717726000},"page":"496-503","source":"Crossref","is-referenced-by-count":33,"title":["Truncated Peroxisome Proliferator-Activated Receptor-\u03b3 Coactivator 1\u03b1 Splice Variant Is Severely Altered in Huntington\u2019s Disease"],"prefix":"10.1159","volume":"8","author":[{"given":"Ashu","family":"Johri","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Anatoly A.","family":"Starkov","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Abhishek","family":"Chandra","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Thomas","family":"Hennessey","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Abhijeet","family":"Sharma","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sara","family":"Orobello","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ferdinando","family":"Squitieri","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lichuan","family":"Yang","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"M. 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