Names & Taxonomy
- Uniprot ID:
- P55789
- Entry Name:
- ALR_HUMAN
- Status:
- reviewed
- Protein Names:
- FAD-linked sulfhydryl oxidase ALR (EC 1.8.3.2) (Augmenter of liver regeneration) (hERV1) (Hepatopoietin)
- Gene Names:
- GFER ALR HERV1 HPO
- Gene Names Primary:
- GFER
- Organism:
- Homo sapiens (Human)
Structure
- Length:
- 205
- Sequence:
- MAAPGERGRFHGGNLFFLPGGARSEMMDDLATDARGRGAGRRDAAASASTPAQAPTSDSPVAEDASRRRPCRACVDFKTWMRTQQKRDTKFREDCPPDREELGRHSWAVLHTLAAYYPDLPTPEQQQDMAQFIHLFSKFYPCEECAEDLRKRLCRNHPDTRTRACFTQWLCHLHNEVNRKLGKPDFDCSKVDERWRDGWKDGSCD
- Proteomes:
- UP000005640
Subcellular location
- Subcellular Location:
- Isoform 1: Mitochondrion intermembrane space.; Isoform 2: Cytoplasm. Secreted.
Function
- Function:
- Isoform 1: FAD-dependent sulfhydryl oxidase that regenerates the redox-active disulfide bonds in CHCHD4/MIA40, a chaperone essential for disulfide bond formation and protein folding in the mitochondrial intermembrane space. The reduced form of CHCHD4/MIA40 forms a transient intermolecular disulfide bridge with GFER/ERV1, resulting in regeneration of the essential disulfide bonds in CHCHD4/MIA40, while GFER/ERV1 becomes re-oxidized by donating electrons to cytochrome c or molecular oxygen.; Isoform 2: May act as an autocrine hepatotrophic growth factor promoting liver regeneration.
- Catalytic Activity:
- 2 R'C(R)SH + O(2) = R'C(R)S-S(R)CR' + H(2)O(2).
- Cofactor:
- COFACTOR: Name=FAD; Xref=ChEBI:CHEBI:57692;
- Cross Reference Drug Bank:
- DB03147
- Gene Ontology Go:
- cytoplasm
extracellular region
mitochondrial intermembrane space
mitochondrion
flavin adenine dinucleotide binding
protein disulfide oxidoreductase activity
thiol oxidase activity
cellular protein metabolic process
protein targeting to mitochondrion - Gene Ontology Biological Process:
- cellular protein metabolic process
protein targeting to mitochondrion - Gene Ontology Molecular Function:
- flavin adenine dinucleotide binding
protein disulfide oxidoreductase activity
thiol oxidase activity - Gene Ontology Cellular Component:
- cytoplasm
extracellular region
mitochondrial intermembrane space
mitochondrion - Keywords:
- 3D-structure
Alternative splicing
Cataract
Complete proteome
Cytoplasm
Disease mutation
Disulfide bond
FAD
Flavoprotein
Growth factor
Mitochondrion
Oxidoreductase
Phosphoprotein
Polymorphism
Reference proteome
Secreted - Interacts With:
- Q9H8W4