RGM-C/Hemojuvelin Antibody

• Reactivity: Mu
• Applications: WB
• Clonality: Polyclonal
• Host: Goat
• Conjugate: Unconjugated
• Concentration: LYOPH

RGM-C/Hemojuvelin Antibody Summary

• Immunogen: Mouse myeloma cell line NS0-derived recombinant mouse RGM-C isoform 1 (R&D Systems, Catalog # 3634-RG)
  Gln33-Asp393 (Ile379Val)
  Accession # Q7TQ32
• Specificity: Detects mouse RGM-C in direct ELISAs and Western blots. In direct ELISAs, approximately 50% cross-reactivity with recombinant human RGM‑C is observed and less than 5% cross-reactivity with recombinant mouse (rm) RGM-A and rmRGM-B is observed.
• Source: N/A
• Isotype: IgG
• Clonality: Polyclonal
• Host: Goat
• Gene: HFE2
• Purity: Immunogen affinity purified
• Purity Statement: Antigen Affinity-purified

Applications/Dilutions

• Dilutions:
  • Western Blot 0.1 ug/mL

Packaging, Storage & Formulations

• Storage: Use a manual defrost freezer and avoid repeated freeze-thaw cycles.
  • 12 months from date of receipt, -20 to -70 °C as supplied.
  • 1 month, 2 to 8 °C under sterile conditions after reconstitution.
  • 6 months, -20 to -70 °C under sterile conditions after reconstitution.
• Buffer: Lyophilized from a 0.2 μm filtered solution in PBS with Trehalose. *Small pack size (SP) is supplied either lyophilized or as a 0.2 µm filtered solution in PBS.
• Preservative: No Preservative
• Concentration: LYOPH
• Purity: Immunogen affinity purified
• Reconstitution Instructions: Reconstitute at 0.2 mg/mL in sterile PBS.

Notes

This product is produced by and ships from R&D Systems, Inc., a Bio-Techne brand.

Alternate Names for RGM-C/Hemojuvelin Antibody

• DL-M
• haemojuvelin
• hemochromatosis type 2 (juvenile)
• Hemojuvelin
• HFE2
• HFE2AMGC23953
• HJV
• HJVHemochromatosis type 2 protein
• JH
• repulsive guidance molecule c
• RGMC
• RGM-C
• RGMCRGM domain family member C

Background

RGM-C, also known as hemojuvelin, is a member of the repulsive guidance molecule (RGM) family of GPI-linked neuronal and muscle membrane glycoproteins (1). RGM‑C is expressed in striated muscle and periportal hepatocytes (2-4). The protein undergoes partial cleavage intracellularly, resulting in a disulfide-linked dimer of the 14 kDa N-terminal and 33 kDa C-terminal portions (3, 5, 6). The N-terminal fragment contains an RGD motif, while the C-terminal fragment carries the GPI attachment site (3, 6). An alternatively spliced isoform lacks the N-terminal fragment. Full length RGM-C can also be released from the cell and circulates in the blood (5, 7). RGM-C is disrupted in type 2A juvenile hemochromatosis, a hereditary iron homeostasis disorder characterized by excessive iron accumulation (4). Loss of RGM-C function results in decreased expression of the iron regulatory hormone hepicidin and increased iron deposition in liver, pancreas, and heart (4, 8). Membrane associated RGM-C upregulates hepicidin while soluble RGM-C downregulates hepicidin expression (7). This appears to be an iron-responsive regulatory system, as high blood iron levels reduce the amount of soluble RGM-C produced (7). RGM-C, similar to RGM-A, associates with neogenin (6). Disease-related point mutations can prevent internal RGM-C cleavage or its ability to interact with neogenin (5, 6). Experimental inflammatory conditions result in decreased RGM-C expression and increased hepicidin expression, although the two effects occur independently (4, 9). RGM-C also functions as a BMP co-receptor and enhances BMP-2 and BMP-4 signaling (10). In this context, RGM-C enhances the BMP-2 upregulation of hepatic hepicidin (10). Mature mouse RGM-C shares 89% and 97% amino acid (aa) sequence identity with human and rat RGM-C, respectively. It shares 51% and 44% aa sequence identity with mouse RGM-A and RGM-B, respectively.

1. Schmidtmer, J. and D. Engelkamp (2004) Gene Exp. Patterns 4:105.
2. Oldekamp, J. et al. (2004) Gene Exp. Patterns 4:283.
3. Niederkofler, V. et al. (2004) J. Neurosci. 24:808.
4. Niederkofler, V. et al. (2005) J. Clin. Invest. 115:2180.
5. Kuninger, D. et al. (2006) J. Cell Sci. 119:3273.
6. Zhang, A.S. et al. (2005) J. Biol. Chem. 280:33885.
7. Lin, L. et al. (2005) Blood 106:2884.
8. Huang, F.W. et al. (2005) J. Clin. Invest. 115:2187.
9. Krijt, J. et al. (2004) Blood 104:4308.
10. Babitt, J.L. et al. (2006) Nat. Genet. 38:531.

Limitations

This product is for research use only and is not approved for use in humans or in clinical diagnosis. Primary Antibodies are guaranteed for 1 year from date of receipt.

Publications for RGM-C/Hemojuvelin Antibody (AF3634)(5)

Publications using AF3634

• J Liu, X Wu, H Wang, J Wei, Q Wu, X Wang, Y Yan, J Cui, J Min, F Wang, J Zhou HFE inhibits type I IFNs signaling by targeting the SQSTM1-mediated MAVS autophagic degradation Autophagy, 2020-08-18;0(0):. 2020-08-18 [PMID: 32746697] (Western Blot, Mouse)
• J Frýdlová, Z Rychtar?ík, I Gurieva, M Vokurka, J Truksa, J Krijt Effect of erythropoietin administration on proteins participating in iron homeostasis in Tmprss6-mutated mask mice PLoS ONE, 2017-10-26;12(10):e0186844. 2017-10-26 [PMID: 29073189] (Western Blot, Mouse)
• I Gurieva, J Frýdlová, Z Rychtar?ík, M Vokurka, J Truksa, J Krijt Erythropoietin administration increases splenic erythroferrone protein content and liver TMPRSS6 protein content in rats Blood Cells Mol. Dis, 2017-02-28;64(0):1-7. 2017-02-28 [PMID: 28282554] (Western Blot, Rat)
• Frydlova J, Prikryl P, Truksa J, Falke L, Du X, Gurieva I, Vokurka M, Krijt J Effect of Erythropoietin, Iron Deficiency and Iron Overload on Liver Matriptase-2 (TMPRSS6) Protein Content in Mice and Rats. PLoS ONE, 2016-02-04;11(2):e0148540. 2016-02-04 [PMID: 26845567] (Western Blot, Mouse)
• Krijt J, Fujikura Y, Ramsay AJ, Velasco G, Necas E Liver hemojuvelin protein levels in mice deficient in matriptase-2 (Tmprss6). Blood Cells Mol. Dis., 2011-05-25;47(2):133-7. 2011-05-25 [PMID: 21612955] (Western Blot, Mouse)

Bioinformatics

• Gene Symbol: HFE2
• Uniprot:
  • Q7TQ32()