Human Hemojuvelin / HFE2 Protein (His Tag)

HFE2A,HJV,JH,RGMC

• 100ug (NPP2180) Please inquiry

Catalog Number	P10410-H08B
Organism Species	Human
Host	Baculovirus-Insect Cells
Synonyms	HFE2A,HJV,JH,RGMC
Molecular Weight	The secreted recombinant human HFE2 consists of 374 amino acids and predicts a molecular mass of 40 kDa. As a result of intracellular cleavage, the apparent molecular mass of the protein is approximately 20, 34 and 44 KDa in SDS-PAGE under reducing conditions, corresponding to the N-terminal, C-terminal portions and the full-length respectively.
predicted N	Gln 36
SDS-PAGE
Purity	> 95 % as determined by SDS-PAGE
Protein Construction	A DNA sequence encoding the mature form of human HFE2 isoform a (Q6ZVN8-1) (Met 1-Ser 399) was fused with a polyhistidine tag at the C-terminus.
Bio-activity
Research Area	Developmental Biology |Metabolism |Pathways and Processes |Vitamins / minerals
Formulation	Lyophilized from sterile PBS, 500mM NaCl, pH 7.0, 10% gly 1. Normally 5 % - 8 % trehalose and mannitol are added as protectants before lyophilization. Specific concentrations are included in the hardcopy of COA.
Background	Hemojuvelin, also known as HFE2, is a membrane-bound and soluble protein which belongs to the repulsive guidance molecule (RGM) family. It is known that RGMs function through Neogenin, a homologue of the Netrin receptor deleted in colon cancer. In mammals, RGM family consists of three glycoproteins which have discrete expression patterns and functions (RGM-A, RGM-B, and RGM-C). Hemojuvelin is expressed in adult and fetal liver, heart, and skeletal muscle. Hemojuvelin acts as a bone morphogenetic protein (BMP) coreceptor. Enhancement of BMP signaling regulates hepcidin (HAMP) expression and iron metabolism. It plays a key role in iron metabolism. Hemojuvelin represents the cellular receptor for hepcidin. It may be a component of the signaling pathway which activates hepcidin or it may act as a modulator of hepcidin expression. Defects in hemojuvelin are the cause of hemochromatosis type 2A, also known as juvenile hemochromatosis (JH).
Reference	Papanikolaou G, et al. (2004) Mutations in HFE2 cause iron overload in chromosome 1q-linked juvenile hemochromatosis. Nat Genet. 36(1):77-82. Babitt JL, et al. (2006) Bone morphogenetic protein signaling by hemojuvelin regulates hepcidin expression. Nat Genet. 38(5):531-9. Zhang AS, et al. (2008) Neogenin-mediated hemojuvelin shedding occurs after hemojuvelin traffics to the plasma membrane. J Biol Chem. 283(25):17494-502.