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SHY1 Shy1p [ Saccharomyces cerevisiae S288C ]

Gene ID: 853009, updated on 4-Jan-2025

Summary

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Official Symbol
SHY1
Official Full Name
Shy1p
Primary source
SGD:S000003344
Locus tag
YGR112W
See related
AllianceGenome:SGD:S000003344; FungiDB:YGR112W; VEuPathDB:YGR112W
Gene type
protein coding
RefSeq status
REVIEWED
Organism
Saccharomyces cerevisiae S288C (strain: S288C)
Lineage
Eukaryota; Fungi; Dikarya; Ascomycota; Saccharomycotina; Saccharomycetes; Saccharomycetales; Saccharomycetaceae; Saccharomyces
Summary
Enables unfolded protein binding activity. Involved in mitochondrial cytochrome c oxidase assembly. Located in mitochondrial inner membrane. Used to study Leigh disease. Human ortholog(s) of this gene implicated in Charcot-Marie-Tooth disease type 4K; Leigh disease; and mitochondrial complex IV deficiency nuclear type 1. Orthologous to human SURF1 (SURF1 cytochrome c oxidase assembly factor). [provided by Alliance of Genome Resources, Jan 2025]
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Genomic context

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See SHY1 in Genome Data Viewer
Location:
chromosome: VII
Exon count:
1
Sequence:
Chromosome: VII; NC_001139.9 (717358..718527)

Chromosome VII - NC_001139.9Genomic Context describing neighboring genes Neighboring gene carboxylic ester hydrolase Neighboring gene uncharacterized protein Neighboring gene Dam1p Neighboring gene chromatin-remodeling histone chaperone SPT6

Genomic regions, transcripts, and products

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Genomic Sequence:
NC_001139.9

Go to nucleotide: Graphics FASTA GenBank

Bibliography

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Related articles in PubMed

  1. SHY1, the yeast homolog of the mammalian SURF-1 gene, encodes a mitochondrial protein required for respiration. Mashkevich G, et al. J Biol Chem, 1997 May 30. PMID 9162072
  2. Shy1p is necessary for full expression of mitochondrial COX1 in the yeast model of Leigh's syndrome. Barrientos A, et al. EMBO J, 2002 Jan 15. PMID 11782424, Free PMC Article
  3. Mss51p promotes mitochondrial Cox1p synthesis and interacts with newly synthesized Cox1p. Perez-Martinez X, et al. EMBO J, 2003 Nov 3. PMID 14592991, Free PMC Article
  4. Shy1p occurs in a high molecular weight complex and is required for efficient assembly of cytochrome c oxidase in yeast. Nijtmans LG, et al. FEBS Lett, 2001 Jun 1. PMID 11389896
  5. Mss51p and Cox14p jointly regulate mitochondrial Cox1p expression in Saccharomyces cerevisiae. Barrientos A, et al. EMBO J, 2004 Sep 1. PMID 15306853, Free PMC Article

See all (60) citations in PubMed

GeneRIFs: Gene References Into Functions

What's a GeneRIF?
  1. Shy1 does not serve as a mobile heme carrier between the heme a synthase and maturing Cox1 but rather cooperates with Cox15 for heme transfer and insertion in early assembly intermediates of cytochrome c oxidase.
    Title: The heme a synthase Cox15 associates with cytochrome c oxidase assembly intermediates during Cox1 maturation.
  2. Exchanging the conserved tyrosine (Y(344)) with aspartate in yeast SHY1 uncouples translational regulation of Cox1 from cytochrome c oxidase assembly.
    Title: Mimicking a SURF1 allele reveals uncoupling of cytochrome c oxidase assembly from translational regulation in yeast.
  3. Analysis of mutations in the SURF1 homolog Shy1 revealed Coa4, a new member of the cytochrome oxidase assembly factor family.
    Title: Analysis of Leigh syndrome mutations in the yeast SURF1 homolog reveals a new member of the cytochrome oxidase assembly factor family.
  4. Shy1 links Cox1 translational regulation to complex IV assembly and supercomplex format
    Title: Shy1 couples Cox1 translational regulation to cytochrome c oxidase assembly.
Submit: New GeneRIF Correction

Interactions

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Products Interactant Other Gene Complex Source Pubs Description

General gene information

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Homology

Gene Ontology Provided by SGD

Function Evidence Code Pubs
enables unfolded protein binding IMP
Inferred from Mutant Phenotype
more info
 PubMed 
Process Evidence Code Pubs
involved_in mitochondrial cytochrome c oxidase assembly IBA
Inferred from Biological aspect of Ancestor
more info
  
involved_in mitochondrial cytochrome c oxidase assembly IMP
Inferred from Mutant Phenotype
more info
 PubMed 
Component Evidence Code Pubs
located_in membrane IDA
Inferred from Direct Assay
more info
 PubMed 
located_in membrane IEA
Inferred from Electronic Annotation
more info
  
located_in mitochondrial inner membrane IDA
Inferred from Direct Assay
more info
 PubMed 
located_in mitochondrial inner membrane IEA
Inferred from Electronic Annotation
more info
  
located_in mitochondrion HDA PubMed 
is_active_in mitochondrion IBA
Inferred from Biological aspect of Ancestor
more info
  
located_in mitochondrion IEA
Inferred from Electronic Annotation
more info
  
located_in peroxisome HDA PubMed 

General protein information

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Preferred Names
Shy1p
NP_011627.1
  • Mitochondrial inner membrane protein required for complex IV assembly; associates with complex IV assembly intermediates and complex III/complex IV supercomplexes; similar to human SURF1 involved in Leigh Syndrome; complex IV is also known as cytochrome c oxidase; also localizes to the peroxisome

NCBI Reference Sequences (RefSeq)

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Genome Annotation

The following sections contain reference sequences that belong to a specific genome build. Explain

Reference assembly

Genomic

  1. NC_001139.9 Reference assembly

    Range
    717358..718527
    Download
    GenBank, FASTA, Sequence Viewer (Graphics)

mRNA and Protein(s)

  1. NM_001181241.1 → NP_011627.1  TPA: Shy1p [Saccharomyces cerevisiae S288C]

    See identical proteins and their annotated locations for NP_011627.1

    Status: REVIEWED

    UniProtKB/Swiss-Prot
    D6VUP5, P53266
    UniProtKB/TrEMBL
    A6ZUC7, B3LIB5, B5VJ57, C7GPS0, C8Z904, G2WEJ6, N1P3N9
    Conserved Domains (1) summary
    COG3346
    Location:58 → 375
    Shy1; Cytochrome oxidase assembly protein ShyY1 [Posttranslational modification, protein turnover, chaperones]
Nucleotide Protein
Heading Accession and Version
Protein Accession Links
GenPept Link UniProtKB Link
P53266.1

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