HFE2 (Hemojuvelin, Hemochromatosis Type 2 Protein, RGM Domain Family Member C, HJV, RGMC, MGC23953)

Size

£685.00

SKU:: 127836
Purity:: Purified by Protein A affinity chromatography.
Storage Conditions:: -20°C
Supplier:: United States Biological
Host:: Mouse
Immunogen:: Partial recombinant protein corresponding to aa93-174 from human HFE2 (NP_973733) with GST tag. MW of the GST tag alone is 26kD.
Clone:: 1C12
Format:: Supplied as a liquid in PBS, pH 7.2.
Extra Details:: 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. Applications: Suitable for use in ELISA, Western Blot and Immunohistochemistry. Other applications not tested. Recommended Dilution: Immunohistochemistry (Formalin fixed paraffin embedded): 3ug/ml Optimal dilutions to be determined by the researcher. AA Sequence: GGCPPSQRLSRSERNRRGAITIDTARRLCKEGLPVEDAYFHSCVFDVLISGDPNFTVAAQAALEDARAFLPDLEKLHLFPS Storage and Stability: May be stored at 4°C for short-term only. Aliquot to avoid repeated freezing and thawing. Store at -20°C. Aliquots are stable for at least 12 months. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.

Shipping Conditions:: Blue Ice