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Shy1p is necessary for full expression of mitochondrial COX1 in the yeast model of Leigh's syndrome
Author(s) -
Barrientos Antoni,
Korr Daniel,
Tzagoloff Alexander
Publication year - 2002
Publication title -
the embo journal
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 7.484
H-Index - 392
eISSN - 1460-2075
pISSN - 0261-4189
DOI - 10.1093/emboj/21.1.43
Subject(s) - columbia university , biology , biological sciences , library science , computational biology , sociology , computer science , media studies
SHY1 codes for a mitochondrial protein required for full expression of cytochrome oxidase (COX) in Saccharomyces cerevisiae . Mutations in the homologous human gene ( SURF1 ) have been reported to cause Leigh's syndrome, a neurological disease associated with COX deficiency. The function of Shy1p/Surf1p is poorly understood. Here we have characterized revertants of shy1 null mutants carrying extragenic nuclear suppressor mutations. The steady‐state levels of COX in the revertants is increased by a factor of 4–5, accounting for their ability to respire and grow on non‐fermentable carbon sources at nearly wild‐type rates. The suppressor mutations are in MSS51 , a gene previously implicated in processing and translation of the COX1 transcript for subunit 1 (Cox1) of COX. The function of Shy1p and the mechanism of suppression of shy1 mutants were examined by comparing the rates of synthesis and turnover of the mitochondrial translation products in wild‐type, mutant and revertant cells. We propose that Shy1p promotes the formation of an assembly intermediate in which Cox1 is one of the partners.