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

Publication

PubMed ID:
15616553 15489334 10712775 8575761 12180965 18691976 18669648 19397338 23186364 24275569 20593814 21383138 22224850 19409522