COX20 (cytochrome c oxidase assembly factor COX20)

2014-11-01  

Identity

HGNC
LOCATION
1q44
LOCUSID
ALIAS
FAM36A,MC4DN11
FUSION GENES

Other Information

Locus ID:

NCBI: 116228
MIM: 614698
HGNC: 26970
Ensembl: ENSG00000203667

Variants:

dbSNP: 116228
ClinVar: 116228
TCGA: ENSG00000203667
COSMIC: COX20

RNA/Proteins

Gene IDTranscript IDUniprot
ENSG00000203667ENST00000366528Q5RI15
ENSG00000203667ENST00000411948Q5RI15
ENSG00000203667ENST00000411948B3KM21

Expression (GTEx)

0
5
10
15
20
25

Pathways

PathwaySourceExternal ID
Gene ExpressionREACTOMER-HSA-74160
Generic Transcription PathwayREACTOMER-HSA-212436
Transcriptional Regulation by TP53REACTOMER-HSA-3700989
TP53 Regulates Metabolic GenesREACTOMER-HSA-5628897
MetabolismREACTOMER-HSA-1430728
The citric acid (TCA) cycle and respiratory electron transportREACTOMER-HSA-1428517
Respiratory electron transport, ATP synthesis by chemiosmotic coupling, and heat production by uncoupling proteins.REACTOMER-HSA-163200
Respiratory electron transportREACTOMER-HSA-611105

Protein levels (Protein atlas)

Not detected
Low
Medium
High

References

Pubmed IDYearTitleCitations
370954812023Clinical and genetic characteristics of children with COX20-associated mitochondrial disorder: case report and literature review.1
370954812023Clinical and genetic characteristics of children with COX20-associated mitochondrial disorder: case report and literature review.1
337510982021Bi-allelic loss of function variants in COX20 gene cause autosomal recessive sensory neuronopathy.6
337510982021Bi-allelic loss of function variants in COX20 gene cause autosomal recessive sensory neuronopathy.6
306561932019Novel pathogenic COX20 variants causing dysarthria, ataxia, and sensory neuropathy.10
310792022019Observation of novel COX20 mutations related to autosomal recessive axonal neuropathy and static encephalopathy.7
306561932019Novel pathogenic COX20 variants causing dysarthria, ataxia, and sensory neuropathy.10
310792022019Observation of novel COX20 mutations related to autosomal recessive axonal neuropathy and static encephalopathy.7
291549482018The mitochondrial TMEM177 associates with COX20 during COX2 biogenesis.21
291549482018The mitochondrial TMEM177 associates with COX20 during COX2 biogenesis.21
242027872014Recessive dystonia-ataxia syndrome in a Turkish family caused by a COX20 (FAM36A) mutation.23
244030532014Human COX20 cooperates with SCO1 and SCO2 to mature COX2 and promote the assembly of cytochrome c oxidase.52
242027872014Recessive dystonia-ataxia syndrome in a Turkish family caused by a COX20 (FAM36A) mutation.23
244030532014Human COX20 cooperates with SCO1 and SCO2 to mature COX2 and promote the assembly of cytochrome c oxidase.52
231252842013A mutation in the FAM36A gene, the human ortholog of COX20, impairs cytochrome c oxidase assembly and is associated with ataxia and muscle hypotonia.42

Citation

Dessen P

COX20 (cytochrome c oxidase assembly factor COX20)

Atlas Genet Cytogenet Oncol Haematol. 2014-11-01

Online version: http://atlasgeneticsoncology.org/gene/62040/cox20-(cytochrome-c-oxidase-assembly-factor-cox20)