Name :
Recombinant Human Hemojuvelin Protein (His Tag)

Biological Activity :

Background :
Hemojuvelin, also known as HFE2, is a membrane-bound and soluble protein that belongs to the repulsive guidance molecule (RGM) family. It is known that RGMs function through Neogenin, a homolog of the Netrin receptor deleted in colon cancer. In mammals, the RGM family consists of three glycoproteins that 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 that 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).

Biological Activity :
Testing in progress

Expression Host :
Human

Source :
Baculovirus-Insect Cells

Tag :

Protein Accession No. :
Q6ZVN8-1

NCBI Gene ID :

Synonyms :

Synonyms :
hemochromatosis type 2 (juvenile)

Amino Acid Sequence :

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.

Purity :
> 95 % as determined by SDS-PAGE

State of Matter :

Product Concentration :

Storage and Stability :
Samples are stable for up to twelve months from date of receipt at -20℃ to -80℃. Store it under sterile conditions at -20℃ to -80℃. It is recommended that the protein be aliquoted for optimal storage. Avoid repeated freeze-thaw cycles.

Endotoxin Level :
< 1.0 EU per μg of the protein as determined by the LAL method

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.

Buffer Solution :
Lyophilized from sterile PBS, 500mM NaCl, pH 7.0, 10% glyPlease contact us for any concerns or special requirements. Normally 5 % – 8 % trehalose, mannitol and 0.01% Tween80 are added as protectants before lyophilization. Please refer to the specific buffer information in the hardcopy of datasheet.

Shipping :
In general, recombinant proteins are provided as lyophilized powder which are shipped at ambient temperature.Bulk packages of recombinant proteins are provided as frozen liquid. They are shipped out with blue ice unless customers require otherwise.

Redissolution :
A hardcopy of datasheet with reconstitution instructions is sent along with the products. Please refer to it for detailed information.

Synonyms :
HFE2A Protein, Human; HJV Protein, Human; JH Protein, Human; RGMC Protein, Human Hemojuvelin 背景信息 Hemojuvelin, also known as HFE2, is a membrane-bound and soluble protein that belongs to the repulsive guidance molecule (RGM) family. It is known that RGMs function through Neogenin, a homolog of the Netrin receptor deleted in colon cancer. In mammals, the RGM family consists of three glycoproteins that 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 that 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).

References & Citations :
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.

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