SURF1 Probably involved in the biogenesis of the COX complex. Defects in SURF1 are a cause of Leigh syndrome (LS). LS is a severe neurological disorder characterized by bilaterally symmetrical necrotic lesions in subcortical brain regions that is commonly associated with systemic cytochrome c oxidase (COX) deficiency. Belongs to the SURF1 family. 2 isoforms of the human protein are produced by alternative splicing. Note: This description may include information from UniProtKB.
Protein type: Membrane protein, integral; Membrane protein, multi-pass; Mitochondrial; Oxidoreductase
Chromosomal Location of Human Ortholog: 9q34.2
Cellular Component:  integral component of membrane; mitochondrial respiratory chain
Molecular Function:  cytochrome-c oxidase activity; protein binding
Biological Process:  aerobic respiration; electron transport chain; mitochondrial respiratory chain complex IV assembly; oxidation-reduction process; oxidative phosphorylation; proton transmembrane transport; respiratory chain complex IV assembly
Disease: Charcot-marie-tooth Disease, Type 4k; Leigh Syndrome
Reference #:  Q15526 (UniProtKB)
Alt. Names/Synonyms: OTTHUMP00000022473; SURF1; surfeit 1; Surfeit locus protein 1
Gene Symbols: SURF1
Molecular weight: 33,331 Da
Basal Isoelectric point: 9.64  Predict pI for various phosphorylation states
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Protein Structure Not Found.

Cross-references to other databases:  STRING  |  cBioPortal  |  Wikipedia  |  neXtProt  |  Protein Atlas  |  BioGPS  |  Pfam  |  Phospho.ELM  |  GeneCards  |  UniProtKB  |  Entrez-Gene  |  GenPept  |  Ensembl Gene  |  Ensembl Protein