SURF1 (SURF1 cytochrome c oxidase assembly factor)

2007-04-01  

Identity

HGNC
LOCATION
9q34.2
LOCUSID
ALIAS
CMT4K,MC4DN1,SHY1

Other Information

Locus ID:

NCBI: 6834
MIM: 185620
HGNC: 11474
Ensembl: ENSG00000148290

Variants:

dbSNP: 6834
ClinVar: 6834
TCGA: ENSG00000148290
COSMIC: SURF1

RNA/Proteins

Gene IDTranscript IDUniprot
ENSG00000148290ENST00000371974Q15526
ENSG00000148290ENST00000371974E5KRX5
ENSG00000148290ENST00000615505A0A087WYS9

Expression (GTEx)

0
10
20
30
40
50
60
70

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
337719872021Defective metabolic programming impairs early neuronal morphogenesis in neural cultures and an organoid model of Leigh syndrome.49
337719872021Defective metabolic programming impairs early neuronal morphogenesis in neural cultures and an organoid model of Leigh syndrome.49
323801622020Novel p.P298L SURF1 mutation in thiamine deficient Leigh syndrome patients compromises cytochrome c oxidase activity.1
323801622020Novel p.P298L SURF1 mutation in thiamine deficient Leigh syndrome patients compromises cytochrome c oxidase activity.1
294818042018Cytochrome C oxydase deficiency: SURF1 gene investigation in patients with Leigh syndrome.2
297151842018Mutations in SURF1 are important genetic causes of Leigh syndrome in Slovak patients.6
299330182018SURF1 mutations in Chinese patients with Leigh syndrome: Novel mutations, mutation spectrum, and the functional consequences.2
294818042018Cytochrome C oxydase deficiency: SURF1 gene investigation in patients with Leigh syndrome.2
297151842018Mutations in SURF1 are important genetic causes of Leigh syndrome in Slovak patients.6
299330182018SURF1 mutations in Chinese patients with Leigh syndrome: Novel mutations, mutation spectrum, and the functional consequences.2
268046542016Tissue- and species-specific differences in cytochrome c oxidase assembly induced by SURF1 defects.13
268046542016Tissue- and species-specific differences in cytochrome c oxidase assembly induced by SURF1 defects.13
227293842013Hypertrophic olivary degeneration on magnetic resonance imaging in mitochondrial syndromes associated with POLG and SURF1 mutations.9
240270612013SURF1 deficiency causes demyelinating Charcot-Marie-Tooth disease.19
227293842013Hypertrophic olivary degeneration on magnetic resonance imaging in mitochondrial syndromes associated with POLG and SURF1 mutations.9

Citation

Dessen P

SURF1 (SURF1 cytochrome c oxidase assembly factor)

Atlas Genet Cytogenet Oncol Haematol. 2007-04-01

Online version: http://atlasgeneticsoncology.org/gene/46853/surf1