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Here we compare the bioenergetic behavior of forebrain mitochondria isolated from different transgenic HD mice (R6\/2, YAC128 and <jats:italic>Hdh<\/jats:italic>150 knock\u2010in) and wild\u2010type littermates with the first determination of <jats:italic>in situ<\/jats:italic> respiratory parameters in intact HD striatal neurons. We assess the Ca<jats:sup>2+<\/jats:sup>\u2010loading capacity of isolated mitochondria by steady Ca<jats:sup>2+<\/jats:sup>\u2010infusion. Mitochondria from R6\/2 mice (12\u201313\u2003weeks) and 12\u2003months YAC128, but not homozygous or heterozygous <jats:italic>Hdh<\/jats:italic>150 knock\u2010in mice (15\u201317\u2003weeks), exhibit increased Ca<jats:sup>2+<\/jats:sup>\u2010loading capacity when compared with respective wild\u2010type littermates. <jats:italic>In situ<\/jats:italic> mitochondria in intact striatal neurons show high respiratory control. Moreover, moderate expression of full\u2010length mutant huntingtin (in <jats:italic>Hdh<\/jats:italic>150 knock\u2010in heterozygotes) does not significantly impair mitochondrial respiration in unstimulated neurons. However, when challenged with energy\u2010demanding stimuli (NMDA\u2010receptor activation in pyruvate\u2010based media to accentuate the mitochondria role in Ca<jats:sup>2+<\/jats:sup>\u2010handling), <jats:italic>Hdh<\/jats:italic>150 neurons are more vulnerable to Ca<jats:sup>2+<\/jats:sup>\u2010deregulation than neurons from their wild\u2010type littermates. These results stress the importance of assessing HD mitochondrial function in the cellular context.<\/jats:p>","DOI":"10.1111\/j.1471-4159.2006.04361.x","type":"journal-article","created":{"date-parts":[[2007,1,13]],"date-time":"2007-01-13T19:17:48Z","timestamp":1168715868000},"page":"241-249","source":"Crossref","is-referenced-by-count":117,"title":["Mitochondrial dysfunction in Huntington\u2019s disease: the bioenergetics of isolated and <i>in situ<\/i> mitochondria from transgenic mice"],"prefix":"10.1111","volume":"101","author":[{"given":"Jorge M. 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