Protein basic information
LiverAtlas Protein ID |
HuLPr15672 |
Uniprot ID |
|
Uniprot Acc |
Q9H3J6;Q8WUC6; |
Protein name |
Probable peptide chain release factor C12orf65, mitochondrial |
Comment |
FUNCTION:May act as a codon-independent translation release factor that has lost all stop codon specificity and directs the termination of translation in mitochondrion (By similarity).||SUBCELLULAR LOCATION:Mitochondrion.||ALTERNATIVE PRODUCTS:Event=Alternative splicing; Named isoforms=2; Name=1; IsoId=Q9H3J6-1; Sequence=Displayed; Name=2; IsoId=Q9H3J6-2; Sequence=VSP 029602, VSP 029603;||DISEASE:Defects in C12orf65 are the cause of combined oxidative phosphorylation deficiency type 7 (COXPD7) [MIM:613559]. A mitochondrial disease resulting in encephalomyopathy. Clinical manifestations include psychomotor delay and regression, ataxia, optic atrophy, nystagmus and muscle atrophy and weakness.||SIMILARITY:Belongs to the prokaryotic/mitochondrial release factor family.||CAUTION:In contrast to other members of the family, lacks the regions that come into close contact with the mRNA in the ribosomal A-site and determine the STOP codon specificity, suggesting a loss of codon specificity |
Subcellular localization |
Mitochondrion. |
Gene name |
|
Protein sequence
|
MSTVGLFHFPTPLTRICPAPWGLRLWEKLTLLSPGIAVTP VQMAGKKDYPALLSLDENELEEQFVKGHGPGGQATNKTS NCVVLKHIPSGIVVKCHQTRSVDQNRKLARKILQEKVDV FYNGENSPVHKEKREAAKKKQERKKRAKETLEKKKLLKE LWESSKKVH |
Database cross reference |
RefSeq Protein accession:NP_001137377
|
Liver relevance
HLPP validation |
Yes/No |
Yes |
Project name |
Chinese Liver;French Liver;Human Fetal Liver; |
Ontology annotation
GO-C |
GO:0005739;C:mitochondrion;NAS:UniProtKB. |
GO-F |
GO:0003747;F:translation release factor activity;IEA:InterPro. |