Western Blot (WB)
(Western Blot: Sample: Rat Liver Tissue.)
Mouse anti-Human Hemojuvelin Monoclonal Antibody | anti-HJV antibody
Monoclonal Antibody to Hemojuvelin (HJV)
Immunocytochemistry in formalin fixed cells: 5-30ug/mL
Immunohistochemistry in formalin fixed frozen section: 5-30ug/mL
Immunohistochemistry in paraffin section: 5-30ug/mL
Optimal working dilutions must be determined by end user.
Western Blot (WB)
(Western Blot: Sample: Rat Liver Tissue.)
Western Blot (WB)
(Western Blot: Sample: Rat Liver Tissue.)
Western Blot (WB)
(Western Blot: Sample: Recombinant HJV, Human.)
Western Blot (WB)
(Western Blot: Sample: Recombinant HJV, Human.)
Immunohistochemistry (IHC)
(DAB staining on IHC-P; Samples: Human Liver Tissue.)
Immunohistochemistry (IHC)
(DAB staining on IHC-P; Samples: Human Liver Tissue.)
Immunohistochemistry (IHC)
(DAB staining on IHC-P; Samples: Human Kidney Tissue)
Immunohistochemistry (IHC)
(DAB staining on IHC-P; Samples: Human Kidney Tissue)
Immunohistochemistry (IHC)
(DAB staining on IHC-P; Samples: Human Glioma Tissue)
Immunohistochemistry (IHC)
(DAB staining on IHC-P; Samples: Human Glioma Tissue)
NCBI and Uniprot Product Information
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. Two uORFs in the 5' UTR negatively regulate the expression and activity of the encoded protein. 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, Oct 2015]
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: cell surface; extracellular space; plasma membrane
Molecular Function: coreceptor activity; protein binding; receptor binding
Biological Process: activin receptor signaling pathway; BMP signaling pathway; cellular iron ion homeostasis; iron ion homeostasis; negative regulation of BMP signaling pathway; negative regulation of transcription from RNA polymerase II promoter; positive regulation of transcription from RNA polymerase II promoter; protein autoprocessing
Disease: Hemochromatosis, Type 2a
Research Articles on HJV
Similar Products
Product Notes
The HJV hfe2 (Catalog #AAA2025747) is an Antibody produced from Mouse and is intended for research purposes only. The product is available for immediate purchase. The Monoclonal Antibody to Hemojuvelin (HJV) reacts with Human and may cross-react with other species as described in the data sheet. AAA Biotech's Hemojuvelin can be used in a range of immunoassay formats including, but not limited to, Western Blot (WB), Immunocytochemistry (ICC), Immunohistochemistry (IHC) Formalin/Paraffin, ELISA (EIA). Western blotting: 0.5-5ug/mL Immunocytochemistry in formalin fixed cells: 5-30ug/mL Immunohistochemistry in formalin fixed frozen section: 5-30ug/mL Immunohistochemistry in paraffin section: 5-30ug/mL Optimal working dilutions must be determined by end user. Researchers should empirically determine the suitability of the HJV hfe2 for an application not listed in the data sheet. Researchers commonly develop new applications and it is an integral, important part of the investigative research process. It is sometimes possible for the material contained within the vial of "Hemojuvelin, Monoclonal Antibody" to become dispersed throughout the inside of the vial, particularly around the seal of said vial, during shipment and storage. We always suggest centrifuging these vials to consolidate all of the liquid away from the lid and to the bottom of the vial prior to opening. Please be advised that certain products may require dry ice for shipping and that, if this is the case, an additional dry ice fee may also be required.Precautions
All products in the AAA Biotech catalog are strictly for research-use only, and are absolutely not suitable for use in any sort of medical, therapeutic, prophylactic, in-vivo, or diagnostic capacity. By purchasing a product from AAA Biotech, you are explicitly certifying that said products will be properly tested and used in line with industry standard. AAA Biotech and its authorized distribution partners reserve the right to refuse to fulfill any order if we have any indication that a purchaser may be intending to use a product outside of our accepted criteria.Disclaimer
Though we do strive to guarantee the information represented in this datasheet, AAA Biotech cannot be held responsible for any oversights or imprecisions. AAA Biotech reserves the right to adjust any aspect of this datasheet at any time and without notice. It is the responsibility of the customer to inform AAA Biotech of any product performance issues observed or experienced within 30 days of receipt of said product. To see additional details on this or any of our other policies, please see our Terms & Conditions page.Item has been added to Shopping Cart
If you are ready to order, navigate to Shopping Cart and get ready to checkout.
