Article
Mutant huntingtin expression induces mitochondrial calcium handling defects in clonal striatal cells: functional consequences.
The Journal of biological chemistry - 17 Nov 2006
Milakovic Tamara, Quintanilla Rodrigo A, Johnson Gail V W
Abstract excerpt
Huntington disease (HD) is caused by a pathological elongation of CAG repeats in the huntingtin protein gene and is characterized by atrophy and neuronal loss primarily in the striatum. Mitochondrial dysfunction and impaired Ca2+ homeostasis in HD have been suggested previously. Here, we elucidate the effects of Ca2+ on mitochondria from the wild type (STHdhQ7/Q7) and mutant (STHdhQ111/Q111) huntingtin-expressing...
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