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Age at onset in Huntingtons disease is modified by the autophagy pathway: implication of the V471A polymorphism in Atg7

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SQOR dynamically regulates H 2 S levels, keeping them within an optimal physiological range: on the one hand, genetic defects (e.g., in Leigh syndrome) or CoQ deficiency that abolish SQOR activity can cause toxic H 2 S accumulation, inhibiting the mitochondrial respiratory chain and leading to severe energy failure