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

Antoni Barrientos, Daniel Korr, Alexander Tzagoloff

Research output: Contribution to journalArticlepeer-review

132 Scopus citations


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.

Original languageEnglish (US)
Pages (from-to)43-52
Number of pages10
JournalEMBO Journal
Issue number1-2
StatePublished - Jan 15 2002
Externally publishedYes


  • Cytochrome oxidase
  • Leigh's syndrome
  • MSS51
  • SHY1
  • SURF1

ASJC Scopus subject areas

  • Genetics
  • Cell Biology


Dive into the research topics of 'Shy1p is necessary for full expression of mitochondrial COX1 in the yeast model of Leigh's syndrome'. Together they form a unique fingerprint.

Cite this