Role of mPTP in mitochondrial damage induced by mutant huntingtin. Representative scheme showing the interplay between calcium regulation, mitochondrial function and mPTP in mutant huntingtin cells. (A), in response to controlled cytosolic calcium increase striatal cells can efficiently regulate calcium homeostasis and mitochondrial function even with the presence of mutant huntingtin. However, pathological calcium increases activated mPTP inducing mitochondrial depolarization and oxidative stress conducing to neuronal dysfunction (B). These two signs of mitochondrial damage can be prevented by inhibition with CsA, an inhibitor of mPTP, which improve mitochondrial function and neuronal health.