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    COQ4 ubiquinone biosynthesis protein COQ4 [ Saccharomyces cerevisiae S288C ]

    Gene ID: 851785, updated on 9-Dec-2024

    Summary

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    Official Symbol
    COQ4
    Official Full Name
    ubiquinone biosynthesis protein COQ4
    Primary source
    SGD:S000002612
    Locus tag
    YDR204W
    See related
    AllianceGenome:SGD:S000002612; FungiDB:YDR204W; VEuPathDB:YDR204W
    Gene type
    protein coding
    RefSeq status
    REVIEWED
    Organism
    Saccharomyces cerevisiae S288C (strain: S288C)
    Lineage
    Eukaryota; Fungi; Dikarya; Ascomycota; Saccharomycotina; Saccharomycetes; Saccharomycetales; Saccharomycetaceae; Saccharomyces
    Summary
    Involved in ubiquinone biosynthetic process. Located in mitochondrial inner membrane. Used to study coenzyme Q10 deficiency disease. Human ortholog(s) of this gene implicated in primary coenzyme Q10 deficiency 7 and spastic ataxia. Orthologous to human COQ4 (coenzyme Q4). [provided by Alliance of Genome Resources, Dec 2024]
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    Genomic context

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    See COQ4 in Genome Data Viewer
    Location:
    chromosome: IV
    Exon count:
    1
    Sequence:
    Chromosome: IV; NC_001136.10 (858137..859144)

    Chromosome IV - NC_001136.10Genomic Context describing neighboring genes Neighboring gene Spc19p Neighboring gene Rav2p Neighboring gene metal cation transporter MSC2 Neighboring gene Ebs1p

    Genomic regions, transcripts, and products

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    Genomic Sequence:
    NC_001136.10

    Go to nucleotide: Graphics FASTA GenBank

    Bibliography

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    Related articles in PubMed

    1. Functional characterization of human COQ4, a gene required for Coenzyme Q10 biosynthesis. Casarin A, et al. Biochem Biophys Res Commun, 2008 Jul 18. PMID 18474229, Free PMC Article
    2. COQ4 mutations cause a broad spectrum of mitochondrial disorders associated with CoQ10 deficiency. Brea-Calvo G, et al. Am J Hum Genet, 2015 Feb 5. PMID 25658047, Free PMC Article
    3. The yeast Coq4 polypeptide organizes a mitochondrial protein complex essential for coenzyme Q biosynthesis. Marbois B, et al. Biochim Biophys Acta, 2009 Jan. PMID 19022396, Free PMC Article
    4. Haploinsufficiency of COQ4 causes coenzyme Q10 deficiency. Salviati L, et al. J Med Genet, 2012 Mar. PMID 22368301, Free PMC Article
    5. Yeast Coq5 C-methyltransferase is required for stability of other polypeptides involved in coenzyme Q biosynthesis. Baba SW, et al. J Biol Chem, 2004 Mar 12. PMID 14701817

    See all (48) citations in PubMed

    GeneRIFs: Gene References Into Functions

    What's a GeneRIF?
    1. Coq4p plays an essential role in organizing a Coq enzyme complex required for Q biosynthesis.
      Title: The yeast Coq4 polypeptide organizes a mitochondrial protein complex essential for coenzyme Q biosynthesis.
    2. study identifies Coq3 peptide and Coq4 peptide as defining members of a Q-biosynthetic Coq polypeptide complex
      Title: Coq3 and Coq4 define a polypeptide complex in yeast mitochondria for the biosynthesis of coenzyme Q.
    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 molecular_function ND
    No biological Data available
    more info
    		  
    Process Evidence Code Pubs
    involved_in ubiquinone biosynthetic process IEA
    Inferred from Electronic Annotation
    more info
    		  
    involved_in ubiquinone biosynthetic process IMP
    Inferred from Mutant Phenotype
    more info
    		 PubMed 
    Component Evidence Code Pubs
    located_in extrinsic component of mitochondrial inner 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
    		  

    General protein information

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    Preferred Names
    ubiquinone biosynthesis protein COQ4
    NP_010490.1
    • Protein with a role in ubiquinone (Coenzyme Q) biosynthesis; possibly functioning in stabilization of Coq7p; located on matrix face of mitochondrial inner membrane; component of a mitochondrial ubiquinone-synthesizing complex; human homolog COQ4 can complement yeast coq4 null mutant

    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_001136.10 Reference assembly

      Range
      858137..859144
      Download
      GenBank, FASTA, Sequence Viewer (Graphics)

    mRNA and Protein(s)

    1. NM_001180512.1 → NP_010490.1  TPA: ubiquinone biosynthesis protein COQ4 [Saccharomyces cerevisiae S288C]

      See identical proteins and their annotated locations for NP_010490.1

      Status: REVIEWED

      UniProtKB/Swiss-Prot
      A6ZYG1, B3LG81, B5VG95, D6VSI5, O13525, Q03454
      UniProtKB/TrEMBL
      C7GMZ5, C8Z5B9, G2WAV1, N1PAF4
      Conserved Domains (1) summary
      pfam05019
      Location:94 → 305
      Coq4; Coenzyme Q (ubiquinone) biosynthesis protein Coq4
    Nucleotide Protein
    Heading Accession and Version
    Protein Accession Links
    GenPept Link UniProtKB Link
    O13525.2

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