Article
Proteolytic processing of OPA1 links mitochondrial dysfunction to alterations in mitochondrial morphology.
The Journal of biological chemistry - 8 Dec 2006
Duvezin-Caubet Stéphane, Jagasia Ravi, Wagener Johannes, Hofmann Sabine, Trifunovic Aleksandra, Hansson Anna, Chomyn Anne, Bauer Matthias F, Attardi Giuseppe, Larsson Nils-Göran, Neupert Walter, Reichert Andreas S
Abstract excerpt
Many muscular and neurological disorders are associated with mitochondrial dysfunction and are often accompanied by changes in mitochondrial morphology. Mutations in the gene encoding OPA1, a protein required for fusion of mitochondria, are associated with hereditary autosomal dominant optic atrophy type I. Here we show that mitochondrial fragmentation correlates with processing of large isoforms of OPA1 in...
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