HJV (Human) Recombinant Protein
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Specification
Product Description
Human HJV (Q6ZVN8-1, Gln33-Asp393) partial recombinant protein with His tag at C-Terminus expressed in HEK293 cells.
Sequence
Gln33-Asp393
Host
Human
Theoretical MW (kDa)
40
Form
Lyophilized
Preparation Method
Mammalian cell (HEK293) expression system
Purity
> 95% as determined by Tris-Bis PAGE; > 95% as determined by HPLC
Endotoxin Level
< 1 EU per 1 ug of protein (determined by LAL method)
Activity
The EC50 was 11.9 ng/mL, messured by ELISA at 0.5 ug/mL.
Quality Control Testing
SEC-HPLC and Tris-Bis PAGE
SEC-HPLC
The purity of Human RGM-C is greater than 95% as determined by SEC-HPLC.
Tris-Bis PAGE
Human RGM-C on Tris-Bis PAGE under reduced condition. The purity is greater than 95%.
Recommend Usage
Biological Activity
ELISA
SDS-PAGE
The optimal working dilution should be determined by the end user.Storage Buffer
Lyophilized from sterile distilled Water is > 100 ug/mL
Storage Instruction
Store at 2°C to 8°C for 1 week. For long term storage, aliquot and store at -20°C to -80°C.
Aliquot to avoid repeated freezing and thawing.Note
Result of bioactivity analysis
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Applications
Enzyme-linked Immunoabsorbent Assay
Immobilized Human RGM-C, His Tag at 0.5 ug/mL (100 uL/well) on the plate. Dose response curve for Anti-RGM-C Antibody, hFc Tag with the EC50 of 11.9 ng/mL determined by ELISA.Functional Study
SDS-PAGE
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Gene Info — HFE2
Entrez GeneID
148738Protein Accession#
Q6ZVN8-1Gene Name
HFE2
Gene Alias
HFE2A, HJV, JH, MGC23953, RGMC
Gene Description
hemochromatosis type 2 (juvenile)
Gene Ontology
HyperlinkGene Summary
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
Other Designations
OTTHUMP00000015582|OTTHUMP00000015583|OTTHUMP00000059680|RGM domain family, member C|hemochromatosis type 2 protein|hemojuvelin
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Disease
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