Article
Characterization of SURF-1 expression and Surf-1p function in normal and disease conditions.
Human molecular genetics - 1 Dec 1999
Tiranti V, Galimberti C, Nijtmans L, Bovolenta S, Perini M P, Zeviani M
Abstract excerpt
Loss-of-function mutations of the SURF-1 gene have been associated with Leigh syndrome with cytochrome c oxidase (COX) deficiency. Mature Surf-1 protein (Surf-1p) is a 30 kDa hydrophobic polypeptide whose function is still unknown. Using antibodies against a recombinant, hemagglutinin-tagged Surf-1p, we have demonstrated that this protein is imported into mitochondria as a larger precursor, which is then...
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