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PMID: 11782424 Published · ppublish English Journal Article Research Support, Non-U.S. Gov't Research Support, U.S. Gov't, P.H.S.

Shy1p is necessary for full expression of mitochondrial COX1 in the yeast model of Leigh's syndrome.

The EMBO journal ·Vol. 21 ·No. 1-2 ·2002-01-15 ·Pages 43-52

Barrientos A, Korr D, Tzagoloff A

Abstract

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.

MeSH Terms
Base Sequence DNA, Fungal/genetics Electron Transport Complex IV/genetics Gene Expression Humans Isoenzymes Leigh Disease/genetics,metabolism Membrane Proteins/genetics,metabolism Mitochondria/enzymology Mitochondrial Proteins Models, Biological Mutation Oxidative Phosphorylation Prostaglandin-Endoperoxide Synthases Protein Biosynthesis Proteins/genetics,metabolism Saccharomyces cerevisiae/genetics,metabolism Saccharomyces cerevisiae Proteins/genetics,metabolism Suppression, Genetic
Chemicals
DNA, Fungal Isoenzymes Membrane Proteins Mitochondrial Proteins Proteins SHY1 protein, S cerevisiae Saccharomyces cerevisiae Proteins Surf-1 protein Prostaglandin-Endoperoxide Synthases Electron Transport Complex IV
Authors & Affiliations
3 authors, click to expand affiliations / ORCID
Barrientos Antoni
Department of Biological Sciences, Columbia University, New York, NY 10027, USA.
Korr Daniel
Tzagoloff Alexander
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Article Info
Journal
The EMBO journal
Abbr.
EMBO J
ISSN
0261-4189
DOI
10.1093/emboj/21.1.43
Published
2002-01-15
Pages
43-52
Language
English
Region
England
NLM ID
8208664
PMCID
PMC125806
Subset
IM
Grants
NIGMS NIH HHS · R01 GM050187 · United States
NIGMS NIH HHS · GM50187 · United States
External Links
PubMed source DOI: 10.1093/emboj/21.1.43 PMC Full Text View journal details
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