Description
Aliases
Augmenter of liver regeneration, hERV1, Hepatopoietin, GFER, ALR, HERV1, HPO
Antibody Type
Polyclonal Antibody
Species
Human
Uniprot ID
P55789
Immunogen
Recombinant human FAD-linked sulfhydryl oxidase ALR protein (81-205AA)
Raised In
Rabbit
Species Reactivity
Human,Mouse
Tested Applications
ELISA, WB, IHC;Recommended dilution:WB:1:200-1000, IHC:1:20-1:200
Background / 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.
Isotype
IgG
Conjugate
Unconjugated
Storage Buffer
PBS with 0.02% sodium azide, 50% glycerol, pH7.3.
Form
Liquid
Storage
Shipped at 4°C. Upon delivery aliquot and store at -20°C or -80°C. Avoid repeated freeze.
Purity
Antigen Affinity Purified
Literature
[1]The mitochondrial disulfide relay system protein GFER is mutated in autosomal-recessive myopathy with cataract and combined respiratory-chain deficiency.” Di Fonzo A., Ronchi D., Lodi T., Fassone E., Tigano M., Lamperti C., Corti S., Bordoni A., Fortunato F., Nizzardo M., Napoli L., Donadoni C., Salani S., Saladino F., Moggio M., Bresolin N., Ferrero I., Comi G.P. Am. J. Hum. Genet. 84:594-604(2009). [2]An electron-transfer path through an extended disulfide relay system: the case of the redox protein ALR.”Banci L., Bertini I., Calderone V., Cefaro C., Ciofi-Baffoni S., Gallo A., Tokatlidis K.J. Am. Chem. Soc. 134:1442-1445(2012). [3]Molecular recognition and substrate mimicry drive the electron-transfer process between MIA40 and ALR.” Banci L., Bertini I., Calderone V., Cefaro C., Ciofi-Baffoni S., Gallo A., Kallergi E., Lionaki E., Pozidis C., Tokatlidis K.Proc. Natl. Acad. Sci. U.S.A. 108:4811-4816(2011).Additional information
| Size | 50μl, 100μl, 50μg, 100μg |
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