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Mutant huntingtin and mitochondrial dysfunction Bossy-Wetzel E; Petrilli A; Knott ABTrends Neurosci 2008[Dec]; 31 (12): 609-16Huntington's disease (HD) is a fatal, inherited neurodegenerative disorder that gradually robs affected individuals of memory, cognitive skills and normal movements. Although research has identified a single faulty gene, the huntingtin gene, as the cause of the disease, a cure remains elusive. Strong evidence indicates that mitochondrial impairment plays a key part in HD pathogenesis. Here, we highlight how mutant huntingtin (mtHtt) might cause mitochondrial dysfunction by either perturbing transcription of nuclear-encoded mitochondrial proteins or by direct interaction with the organelle and modulation of respiration, mitochondrial membrane potential and Ca(2+) buffering. In addition, we propose that mtHtt might convey its neurotoxicity by evoking defects in mitochondrial dynamics, organelle trafficking and fission and fusion, which, in turn, might result in bioenergetic failure and HD-linked neuronal dysfunction and cell death. Finally, we speculate how mitochondria might dictate selective vulnerability of long projection neurons, such as medium spiny neurons, which are particularly affected in HD.|*Mutation[MESH]|Animals[MESH]|Humans[MESH]|Huntingtin Protein[MESH]|Mitochondria/pathology/physiology[MESH]|Mitochondrial Diseases/*genetics[MESH]|Models, Biological[MESH]|Nerve Tissue Proteins/*genetics[MESH]|Nuclear Proteins/*genetics[MESH] |
