• Alternative Names :
• FAD-linked sulfhydryl oxidase ALR (EC 1.8.3.2) (Augmenter of liver regeneration) (hERV1) (Hepatopoietin), GFER, ALR HERV1 HPO

• Categories :
• Primary Antibodies

• Clonality :
• polyclonal

• Description :
• 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.

• Host :
• Rabbit

• Immunogen :
• Recombinant Human FAD-linked sulfhydryl oxidase ALR protein (81-205AA)

• Involvement In Disease :
• Myopathy, mitochondrial progressive, with congenital cataract, hearing loss and developmental delay (MPMCHD)

• Isotype :
• IgG

• Raised In :
• Rabbit

• Reactivity :
• Human, Mouse

• Regulatory :
• RUO

• Relevance :
• 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.

• Species :
• Homo Sapiens (Human)

• Subcellular Location :
• Isoform 1: Mitochondrion intermembrane space, SUBCELLULAR LOCATION: Isoform 2: Cytoplasm, Secreted

• Antigen :
• GFER

• Uniprot :
• P55789

• Gene Id :
• 2671

• Database Links :
• 
  

  HGNC:4236

  OMIM:600924

  KEGG:hsa:2671

  STRING:9606.ENSP00000248114

  UniGene:Hs.27184

• Function :
• Isoform 1

• Research Area :
• Signal Transduction

• Tissue Specificity :
• Ubiquitously expressed. Highest expression in the testis and liver and low expression in the muscle.

• Weight :
• 15,029 Da

• Buffer :
• PBS with 0.02% sodium azide, 50% glycerol, pH7.3.

• Form :
• Liquid

• Format :
• Liquid

• Purification :
• Antigen Affinity Purified

• Purity :
• Antigen Affinity Purified

• Storage :
• Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.

• Storage Buffer :
• 
  
  PBS with 0.02% sodium azide, 50% glycerol, pH7.3.

• Datasheet :
• 

• ELISA Protocol :

• Western Blotting(WB) Protocol :

• Immunohistochemistry (IHC) Protocol :