Hemojuvelin (HJV) Recombinant Protein | HJV recombinant protein

Recombinant Hemojuvelin (HJV)

Cat. Num. AAA2011803

• Gene Names: HFE2; JH; HJV; RGMC; HFE2A
• Applications: SDS-Page, Western Blot, ELISA, Immunoprecipitation
• Purity: > 95%
• Synonyms: Hemojuvelin (HJV); Recombinant Hemojuvelin (HJV); HJV recombinant protein

• Host: E Coli
• Purity/Purification: > 95%
• Form/Format: Supplied as lyophilized form in PBS,pH7.4, containing 5% sucrose, 0.01% sarcosyl.
• Sequence: The target protein is fused with N-terminal His-Tag, its sequence is listed below.; MGHHHHHHSGSEF-KVYQAEVDNL PVAFEDGSIN GGDRPGGSSL SIQTANPGNH VEIQAAYIGT TIIIRQTAGQ LSFSIKVAED VAMAFSAEQD LQLCVGGCPP SQRLSRSERN RRGAITIDTA RRLCKEGLPV EDAYFHSCVF DVLISGDPNF TVAAQAALED ARAFLPDLEK LHLFPSDAGV PLSSATLLAP LLS
• Sequence Length: 313

• Applicable Applications for HJV recombinant protein: SDS-PAGE, Western Blot (WB), ELISA (EIA), Immunoprecipitation (IP)
• Organism: Homo sapiens (Human)
• Expression System: Prokaryotic expression
• Residues: Lys234~Ser416 (Accession # Q6ZVN8) with N-terminal His-Tag
• Endotoxin Level: <1.0EU per 1ug (determined by the LAL method)
• Reconstitution: Reconstitute in sterile PBS, pH7.2-pH7.4.
• Preparation and Storage: Avoid repeated freeze/thaw cycles. Store at 2-8 degree C for one month. Aliquot and store at -80 degree C for 12 months.; Stability Test: The thermal stability is described by the loss rate of the targetprotein. The loss rate was determined by accelerated thermal degradation test,that is, incubate the protein at 37 degree C for 48h, and no obvious degradation andprecipitation were observed. (Referring from China Biological Products Standard,which was calculated by the Arrhenius equation.) The loss of this protein is lessthan 5% within the expiration date under appropriate storage condition.

Sequence Information

SDS-Page

Testing Data

NCBI and Uniprot Product Information

• NCBI GI #: 44662823
• NCBI GeneID: 148738
• NCBI Accession #: NP_660320.3
• NCBI GenBank Nucleotide #: NM_145277.4
• UniProt Accession #: Q6ZVN8; Q2PQ63; Q6IMF6; Q8NAH2; Q8WVJ5; B1ALI7
• Molecular Weight: 22kDa
• NCBI Official Full Name: hemojuvelin isoform b
• NCBI Official Synonym Full Names: hemochromatosis type 2 (juvenile)
• NCBI Official Symbol: HFE2
• NCBI Official Synonym Symbols: JH; HJV; RGMC; HFE2A
• NCBI Protein Information: hemojuvelin; haemojuvelin; RGM domain family member C; repulsive guidance molecule c; hemochromatosis type 2 protein
• UniProt Protein Name: Hemojuvelin
• UniProt Gene Name: HFE2
• UniProt Synonym Gene Names: HJV; RGMC
• UniProt Entry Name: RGMC_HUMAN

NCBI Description

The product of this gene is involved in iron metabolism. It may be a component of the signaling pathway which activates hepcidin or it may act as a modulator of hepcidin expression. It could also represent the cellular receptor for hepcidin. Alternatively spliced transcript variants encoding different isoforms have been identified for this gene. Defects in this gene are the cause of hemochromatosis type 2A, also called juvenile hemochromatosis (JH). JH is an early-onset autosomal recessive disorder due to severe iron overload resulting in hypogonadotrophic hypogonadism, hepatic fibrosis or cirrhosis and cardiomyopathy, occurring typically before age of 30. [provided by RefSeq, Jul 2008]

Uniprot Description

HFE2: Member of the repulsive guidance molecule (RGM) family. Involved in iron metabolism. Acts as a bone morphogenetic protein (BMP) coreceptor. Enhancement of BMP signaling regulates hepcidin (HAMP) expression and iron metabolism. May cooperate with hepcidin to restrict iron absorption in the gut. Could represent the cellular receptor for hepcidin. Defects in HFE2 are the cause of hemochromatosis type 2A (HFE2A); also known as juvenile hemochromatosis (JH). HFE2A is an early-onset autosomal recessive disorder due to severe iron overload resulting in hypogonadotrophic hypogonadism, hepatic fibrosis or cirrhosis and cardiomyopathy, occurring typically before age of 30. It is the consequence of intestinal iron hyperabsorption associated with macrophages that do not load iron. Deleterious mutations of HFE2 reduced HAMP (hepcidin) levels despite iron overload, which normally induces HAMP expression. Belongs to the repulsive guidance molecule (RGM) family. 3 isoforms of the human protein are produced by alternative splicing.

Protein type: Membrane protein, GPI anchor

Chromosomal Location of Human Ortholog: 1q21.1

Cellular Component: extracellular space; cell surface; plasma membrane

Molecular Function: protein binding; coreceptor activity

Biological Process: BMP signaling pathway; axon guidance; iron ion homeostasis; positive regulation of transcription from RNA polymerase II promoter

Disease: Hemochromatosis, Type 2a