[HTML][HTML] Mitochondrial respiration and ATP production are significantly impaired in striatal cells expressing mutant huntingtin
T Milakovic, GVW Johnson - Journal of Biological Chemistry, 2005 - ASBMB
There is significant evidence that energy production impairment and mitochondrial
dysfunction play a role in the pathogenesis of Huntington disease. Nonetheless, the specific
mitochondrial defects due to the presence of mutant huntingtin have not been fully
elucidated. To determine the effects of mutant huntingtin on mitochondrial energy
production, a thorough analysis of respiration, ATP production, and functioning of the
respiratory complexes was carried out in clonal striatal cells established from Hdh Q7 (wild …
dysfunction play a role in the pathogenesis of Huntington disease. Nonetheless, the specific
mitochondrial defects due to the presence of mutant huntingtin have not been fully
elucidated. To determine the effects of mutant huntingtin on mitochondrial energy
production, a thorough analysis of respiration, ATP production, and functioning of the
respiratory complexes was carried out in clonal striatal cells established from Hdh Q7 (wild …