Huntington's disease (HD) is caused by an expansion of exonic CAG triplet repeats in the
gene encoding huntingtin protein (Htt), but the mechanisms by which this mutant protein
causes neurodegeneration remain unknown. Here we show that lymphoblast mitochondria
from patients with HD have a lower membrane potential and depolarize at lower calcium
loads than do mitochondria from controls. We found a similar defect in brain mitochondria
from transgenic mice expressing full-length mutant huntingtin, and this defect preceded the …

Early mitochondrial calcium defects in Huntington's disease are a direct effect of
polyglutamines | CiNii Research … Early mitochondrial calcium defects in Huntington's
disease are a direct effect of polyglutamines …