COQ4 (coenzyme Q4)

2014-11-01  

Identity

HGNC
LOCATION
9q34.11
LOCUSID
ALIAS
CGI-92,COQ10D7
FUSION GENES

Other Information

Locus ID:

NCBI: 51117
MIM: 612898
HGNC: 19693
Ensembl: ENSG00000167113

Variants:

dbSNP: 51117
ClinVar: 51117
TCGA: ENSG00000167113
COSMIC: COQ4

RNA/Proteins

Gene IDTranscript IDUniprot
ENSG00000167113ENST00000300452Q9Y3A0
ENSG00000167113ENST00000372875Q5T4B9
ENSG00000167113ENST00000608951V9GY32
ENSG00000167113ENST00000609948V9GZ09

Expression (GTEx)

0
10
20
30
40
50
60
70

Protein levels (Protein atlas)

Not detected
Low
Medium
High

References

Pubmed IDYearTitleCitations
380136262024Biallelic variants in the COQ4 gene caused hereditary spastic paraplegia predominant phenotype.1
380144832024Biallelic COQ4 Variants in Hereditary Spastic Paraplegia: Clinical and Molecular Characterization.1
382958032024COQ4 is required for the oxidative decarboxylation of the C1 carbon of coenzyme Q in eukaryotic cells.2
380136262024Biallelic variants in the COQ4 gene caused hereditary spastic paraplegia predominant phenotype.1
380144832024Biallelic COQ4 Variants in Hereditary Spastic Paraplegia: Clinical and Molecular Characterization.1
382958032024COQ4 is required for the oxidative decarboxylation of the C1 carbon of coenzyme Q in eukaryotic cells.2
379489952023Epilepsy and Coenzyme Q10 deficiency with COQ4 variants.0
379489952023Epilepsy and Coenzyme Q10 deficiency with COQ4 variants.0
360476082022Bi-Allelic COQ4 Variants Cause Adult-Onset Ataxia-Spasticity Spectrum Disease.4
360476082022Bi-Allelic COQ4 Variants Cause Adult-Onset Ataxia-Spasticity Spectrum Disease.4
332158592021Clinical spectrum in multiple families with primary COQ(10) deficiency.10
337045552021New pathogenic variants in COQ4 cause ataxia and neurodevelopmental disorder without detectable CoQ(10) deficiency in muscle or skin fibroblasts.15
332158592021Clinical spectrum in multiple families with primary COQ(10) deficiency.10
337045552021New pathogenic variants in COQ4 cause ataxia and neurodevelopmental disorder without detectable CoQ(10) deficiency in muscle or skin fibroblasts.15
306592642019Clinical phenotype, in silico and biomedical analyses, and intervention for an East Asian population-specific c.370G>A (p.G124S) COQ4 mutation in a Chinese family with CoQ10 deficiency-associated Leigh syndrome.11

Citation

Dessen P

COQ4 (coenzyme Q4)

Atlas Genet Cytogenet Oncol Haematol. 2014-11-01

Online version: http://atlasgeneticsoncology.org/gene/62026/coq4-(coenzyme-q4)