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GFER
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. Ubiquitously expressed. Highest expression in the testis and liver and low expression in the muscle. 2 alternatively spliced human isoforms have been reported. Note: This description may include information from UniProtKB.
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| Protein type: EC 1.8.3.2; Mitochondrial; Oxidoreductase |
| Chromosomal Location of human Ortholog: 16p13.3 |
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Cellular Component:
cytosol; extracellular space; mitochondrial intermembrane space; mitochondrion
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Molecular Function:
flavin adenine dinucleotide binding; flavin-dependent sulfhydryl oxidase activity; growth factor activity; protein binding; protein-disulfide reductase activity
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Biological Process:
cellular response to actinomycin D; cellular response to lipopolysaccharide; cellular response to toxic substance; cellular response to tumor necrosis factor; liver development; liver regeneration; negative regulation of apoptotic process; negative regulation of natural killer cell mediated cytotoxicity; positive regulation of DNA biosynthetic process; signal transduction
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Disease: Myopathy, Mitochondrial Progressive, With Congenital Cataract, Hearing Loss, And Developmental Delay
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Reference #:
P55789
(UniProtKB)
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Alt. Names/Synonyms: ALR; Augmenter of liver regeneration; ERV1; ERV1 homolog; erv1-like growth factor; FAD-linked sulfhydryl oxidase ALR; GFER; growth factor, augmenter of liver regeneration; hepatic regenerative stimulation substance; Hepatopoietin; hepatopoietin protein; HERV1; HPO; HPO1; HPO2; HSS; MMCHD; MPMCD; truncated augmenter of liver regeneration
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Gene Symbols: GFER
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Molecular weight:
23,449 Da
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Basal Isoelectric point:
7.57
Predict pI for various phosphorylation states
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