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Gene Review

SHY1  -  Shy1p

Saccharomyces cerevisiae S288c

Synonyms: Cytochrome oxidase assembly protein SHY1, G6150, SURF1 homolog of Yeast, SURF1-like protein, YGR112W
 
 
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Disease relevance of SHY1

  • We studied SHY1, the yeast homologue of SURF1, with an aim to obtain a better understanding of the molecular pathogenesis of cytochrome c oxidase (COX) deficiency in SURF1 mutant cells from Leigh syndrome patients [1].
 

High impact information on SHY1

  • The post-transcriptional role of Mss51p in mitochondrial gene expression is of great interest since MSS51 mutations suppress the respiratory defect caused by shy1 mutations [2].
  • Here we have characterized revertants of shy1 null mutants carrying extragenic nuclear suppressor mutations [3].
  • Assembly of COX was analysed in a shy1 null mutant strain by two-dimensional polyacrylamide gel electrophoresis (2D-PAGE) [1].
 

Analytical, diagnostic and therapeutic context of SHY1

  • Protein interactions of both Shy1p and Surf1p are implied by their appearance in a high molecular weight complex of about 250 kDa, as shown by 2D-PAGE [1].

References

  1. Shy1p occurs in a high molecular weight complex and is required for efficient assembly of cytochrome c oxidase in yeast. Nijtmans, L.G., Artal Sanz, M., Bucko, M., Farhoud, M.H., Feenstra, M., Hakkaart, G.A., Zeviani, M., Grivell, L.A. FEBS Lett. (2001) [Pubmed]
  2. Mss51p promotes mitochondrial Cox1p synthesis and interacts with newly synthesized Cox1p. Perez-Martinez, X., Broadley, S.A., Fox, T.D. EMBO J. (2003) [Pubmed]
  3. Shy1p is necessary for full expression of mitochondrial COX1 in the yeast model of Leigh's syndrome. Barrientos, A., Korr, D., Tzagoloff, A. EMBO J. (2002) [Pubmed]
 
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