Hephaestin (Hp)

Elemental iron is required for a variety of normal cellular functions and vital for proper growth and development. However, natural iron is quite insoluble and excess iron is harmful, since it can catalyze the formation of potentially damaging reactive oxygen species. Humans also have very limited capacity to excrete iron. Therefore, cells have developed mechanisms to improve solubility of iron and to control intracellular iron levels at the point of absorption in the intestine and other tissue. Several proteins including Ferritin, transferrin (Tf), transferrin receptors (TfRs), and iron regulatory proteins (IRPs), iron transporter (NRMAP2/DMT1/DCT1) etc play a key role in iron metabolism. Some genes involved in iron-metabolism are associated with genetic disorders such as Friedreich's Ataxia (Frataxin), genetic hemochromatosis (HFE), and Sex-linked anemia (Hephaestin). Hephaestin (Hp) gene was originally cloned as the gene defective in sex-linked anemia (sla) that is characterized by moderate to severe microcytic hypochronic anemia. Hp protein (mouse/rat 1157 aa, human 1158 aa, ~155kD) encodes a single-membrane spanning domain protein with extensive homology (50-60%) with copper containing serum ferrooxidase ceruloplasmin (Cp). TM domain is located very close to the C-temrinus followed by the short cytoplasmic tail. Cp is involved in release of iron from various tissues. Hp is mostly expressed throughout the small intestine and colon. Low levels are also found in some other tissues (spleen, placenta and lung). It is localized in mature villus enterocytes with little or no expression in the crypts. Hp levels are not affected by iron status.\n\nApplications: \nSuitable for use in ELISA, Western Blot and Immunohistochemistry. Other applications not tested.\n\nRecommended Dilution:\nWestern Blot: 1-10ug/ml using ECL.\nELISA: 1:100,000 using 50-100ng H2030-05 Control peptide/well.\nImmunohistochemistry: See citation\nOptimal dilutions to be determined by the researcher.\n\nStorage and Stability:\nMay be stored at 4 degrees C for short-term only. Aliquot to avoid repeated freezing and thawing. Store at -20 degrees C. Aliquots are stable for at least 12 months. For maximum recovery of product, centrifuge the original vial prior to removing the cap.

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SPECIFICATIONS

Catalog Number

H2030-06

Size

50ug

Applications

ELISA, IHC, WB

Hosts

Rabbit

Reactivities

Mouse

Form

Supplied as a liquid in PBS, pH 7.4, 0.1% BSA.

P Type

Pab

Purity

Purified by immunoaffinity chromatography.

Isotype

IgG

References

1. Hahn P 2004 Proc. Natl. Acad. Sci. 101: 13850 - 13855 (IHC). 2. Vulpe CD et al (1999) Nat. genet. 21, 195-199; 3. Frazer DM et al (2001) Am. J. Physiol. 281, G931-G939; (review).

Additional Info

Recognizes mouse Hephaestin.