Gene Symbol: SHY1
Description: Shy1p
Alias: Shy1p
Species: Saccharomyces cerevisiae S288c
Products:     SHY1

Top Publications

  1. Nijtmans L, Artal Sanz M, Bucko M, Farhoud M, Feenstra M, Hakkaart G, et al. Shy1p occurs in a high molecular weight complex and is required for efficient assembly of cytochrome c oxidase in yeast. FEBS Lett. 2001;498:46-51 pubmed
    ..The presence of a significant amount of holo-COX in the SHY1-disruptant strain suggests that Shy1p may either facilitate assembly of the enzyme, or increase its stability...
  2. Mick D, Wagner K, van der Laan M, Frazier A, Perschil I, Pawlas M, et al. Shy1 couples Cox1 translational regulation to cytochrome c oxidase assembly. EMBO J. 2007;26:4347-58 pubmed
    ..Defects in the assembly process lead to severe human disorders such as Leigh syndrome. Shy1 is an assembly factor for complex IV in Saccharomyces cerevisiae and mutations of its human homolog, SURF1, are the ..
  3. Mashkevich G, Repetto B, Glerum D, Jin C, Tzagoloff A. SHY1, the yeast homolog of the mammalian SURF-1 gene, encodes a mitochondrial protein required for respiration. J Biol Chem. 1997;272:14356-64 pubmed
    ..An antibody against the carboxyl-terminal half of Shy1p has been used to localize the protein in the inner mitochondrial membrane...
  4. Barrientos A, Zambrano A, Tzagoloff A. Mss51p and Cox14p jointly regulate mitochondrial Cox1p expression in Saccharomyces cerevisiae. EMBO J. 2004;23:3472-82 pubmed
    ..The release of Mss51p from the complex occurs at a downstream step in the assembly pathway, probably catalyzed by Shy1p.
  5. Pierrel F, Bestwick M, Cobine P, Khalimonchuk O, Cricco J, Winge D. Coa1 links the Mss51 post-translational function to Cox1 cofactor insertion in cytochrome c oxidase assembly. EMBO J. 2007;26:4335-46 pubmed
    ..Respiration in coa1Delta and shy1Delta cells is enhanced when Mss51 and Cox10 are coexpressed. Shy1 has been implicated in formation of the heme a3-Cu(B) site in Cox1...
  6. Fontanesi F, Jin C, Tzagoloff A, Barrientos A. Transcriptional activators HAP/NF-Y rescue a cytochrome c oxidase defect in yeast and human cells. Hum Mol Genet. 2008;17:775-88 pubmed
    ..In the yeast Saccharomyces cerevisiae, deletion of the SURF1 homologue SHY1 results in a similar COX deficiency...
  7. Bestwick M, Khalimonchuk O, Pierrel F, Winge D. The role of Coa2 in hemylation of yeast Cox1 revealed by its genetic interaction with Cox10. Mol Cell Biol. 2010;30:172-85 pubmed publisher
    ..of coa2 Delta cells but lacks suppressor activity for two other CcO assembly mutant strains, the coa1 Delta and shy1 Delta mutants...
  8. Bestwick M, Jeong M, Khalimonchuk O, Kim H, Winge D. Analysis of Leigh syndrome mutations in the yeast SURF1 homolog reveals a new member of the cytochrome oxidase assembly factor family. Mol Cell Biol. 2010;30:4480-91 pubmed publisher
    Three missense SURF1 mutations identified in patients with Leigh syndrome (LS) were evaluated in the yeast homolog Shy1 protein...
  9. Pierrel F, Khalimonchuk O, Cobine P, Bestwick M, Winge D. Coa2 is an assembly factor for yeast cytochrome c oxidase biogenesis that facilitates the maturation of Cox1. Mol Cell Biol. 2008;28:4927-39 pubmed publisher
    ..Coa1 and Shy1 function at an early step in maturation of the Cox1 subunit of CcO...

More Information


  1. Kucejova B, Li L, Wang X, Giannattasio S, Chen X. Pleiotropic effects of the yeast Sal1 and Aac2 carriers on mitochondrial function via an activity distinct from adenine nucleotide transport. Mol Genet Genomics. 2008;280:25-39 pubmed publisher
    ..Thus, our data support the view that the V function is independent of adenine nucleotide transport associated with Sal1p and Aac2p and this evolutionarily conserved activity affects multiple processes in mitochondria. ..
  2. Fontanesi F, Clemente P, Barrientos A. Cox25 teams up with Mss51, Ssc1, and Cox14 to regulate mitochondrial cytochrome c oxidase subunit 1 expression and assembly in Saccharomyces cerevisiae. J Biol Chem. 2011;286:555-66 pubmed publisher
    ..This step is probably catalyzed by assembly chaperones such as Shy1 in a process coupled to the release of Ssc1-Mss51 from the complex...
  3. Bareth B, Dennerlein S, Mick D, Nikolov M, Urlaub H, Rehling P. The heme a synthase Cox15 associates with cytochrome c oxidase assembly intermediates during Cox1 maturation. Mol Cell Biol. 2013;33:4128-37 pubmed publisher
    ..However, at which step of the assembly process and how heme is inserted into Cox1 have remained an enigma. Shy1, the yeast SURF1 homolog, has been implicated in heme transfer to Cox1, whereas the heme a synthase, Cox15, ..
  4. Bareth B, Nikolov M, Lorenzi I, Hildenbeutel M, Mick D, Helbig C, et al. Oms1 associates with cytochrome c oxidase assembly intermediates to stabilize newly synthesized Cox1. Mol Biol Cell. 2016;27:1570-80 pubmed publisher
    ..Our analyses show that Oms1 participates in cytochrome c oxidase assembly by stabilizing newly synthesized Cox1. ..
  5. Fontanesi F, SOTO I, Horn D, Barrientos A. Mss51 and Ssc1 facilitate translational regulation of cytochrome c oxidase biogenesis. Mol Cell Biol. 2010;30:245-59 pubmed publisher
    ..We conclude that Ssc1, by interacting with Mss51 and Mss51-containing complexes, plays a critical role in Cox1 biogenesis, COX assembly, and the translational regulation of these processes. ..
  6. Mick D, Vukotic M, Piechura H, Meyer H, Warscheid B, Deckers M, et al. Coa3 and Cox14 are essential for negative feedback regulation of COX1 translation in mitochondria. J Cell Biol. 2010;191:141-54 pubmed publisher
    ..Our data indicate that Coa1 binding to sequestered Mss51 in complex with Cox14, Coa3, and Cox1 is essential for full inactivation. ..
  7. Reinhold R, Bareth B, Balleininger M, Wissel M, Rehling P, Mick D. Mimicking a SURF1 allele reveals uncoupling of cytochrome c oxidase assembly from translational regulation in yeast. Hum Mol Genet. 2011;20:2379-93 pubmed publisher
    ..SURF1, termed Shy1 in Saccharomyces cerevisiae, is a conserved assembly factor for the terminal enzyme of the respiratory chain, ..
  8. Khalimonchuk O, Jeong M, Watts T, Ferris E, Winge D. Selective Oma1 protease-mediated proteolysis of Cox1 subunit of cytochrome oxidase in assembly mutants. J Biol Chem. 2012;287:7289-300 pubmed publisher
    ..Specific Cox1 proteolysis in coa2? cells arises from a combination of Oma1 activation and a susceptible conformation of Cox1. ..