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    SHY1 Shy1p [ Saccharomyces cerevisiae S288C ]

    Gene ID: 853009, updated on 9-Dec-2024

    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, Dec 2024]
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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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