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COQ4 ubiquinone biosynthesis protein COQ4 [ Saccharomyces cerevisiae S288C ]

Gene ID: 851785, updated on 28-Oct-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, Oct 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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