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Hemojuvelin Cell Lysate | HJV cell lysate

Human Hemojuvelin Baculovirus-Insect cells Overexpression Lysate

Gene Names
HFE2; JH; HJV; RGMC; HFE2A
Applications
Western Blot
Synonyms
Hemojuvelin; Human Hemojuvelin Baculovirus-Insect cells Overexpression Lysate; Human Hemojuvelin/HFE2 Insect Cell Lysate (WB positive control); hemochromatosis type 2 (juvenile); Human HFE2A Overexpression Lysate; Human HJV Overexpression Lysate; Human JH Overexpression Lysate; Human RGMC Overexpression Lysate; HJV cell lysate
Ordering
For Research Use Only!
Host
Baculovirus-Insect cells
Form/Format
1x Sample Buffer (1x modified RIPA buffer+1x SDS loading buffer).
Modified RIPA Lysis Buffer: 50 mM Tris-HCl pH 7.4, 150 mM NaCl, 1mM EDTA, 1% Triton X-100, 0.1% SDS, 1% Sodium deoxycholate, 1mM PMSF.
Sequence Positions
374aa
Sequence
Met1-Ser399
Applicable Applications for HJV cell lysate
Western Blot (WB)
Application Notes
WB: Use at an assay dependent dilution.
Species
Human
Recommend Usage
1. Centrifuge the tube for a few seconds and ensure the pellet at the bottom of the tube.2. Re-dissolve the pellet using 200uL pure water and boil for 2-5 min.
Sequence Construction
A DNA sequence encoding the mature form of human HFE2 isoform a (Q6ZVN8-1) (Met 1-Ser 399) was fused with a polyhistidine tag at the C-terminus.
Preparation Method
Cell lysate was prepared by homogenization of the over-expressed cells in ice-cold modified RIPA Lysis Buffer with cocktail of protease inhibitors. Cell debris was removed by centrifugation. Protein concentration was determined by Bradford assay (protein assay, Microplate Standard assay). The cell lysate was boiled for 5 min in 1 x SDS loading buffer (50mM Tris-HCl pH 6.8, 12.5% glycerol, 1% sodium dodecylsulfate, 0.01% bromophenol blue) containing 5% b-mercaptoethanol, and lyophilized.
Preparation and Storage
Store at 4 degree C for up to twelve months from date of receipt. After re-dissolution, aliquot and store at -80 degree C for up to twelve months. Avoid repeated freeze-thaw cycles.
Samples are stable for up to twelve months from date of receipt.
Related Product Information for HJV cell lysate
Hemojuvelin, also known as HFE2, is a membrane-bound and soluble protein that belongs to the repulsive guidance molecule (RGM) family. It is known that RGMs function through Neogenin, a homolog of the Netrin receptor deleted in colon cancer. In mammals, the RGM family consists of three glycoproteins that have discrete expression patterns and functions (RGM-A, RGM-B, and RGM-C). Hemojuvelin is expressed in adult and fetal liver, heart, and skeletal muscle. Hemojuvelin acts as a bone morphogenetic protein (BMP) coreceptor. Enhancement of BMP signaling regulates hepcidin (HAMP) expression and iron metabolism. It plays a key role in iron metabolism. Hemojuvelin represents the cellular receptor for hepcidin. It may be a component of the signaling pathway that activates hepcidin or it may act as a modulator of hepcidin expression. Defects in hemojuvelin are the cause of hemochromatosis type 2A, also known as juvenile hemochromatosis (JH).

This Human Hemojuvelin overexpression lysate was created in Baculovirus-Insect cells and intented for use as a Western blot (WB) positive control. Purification of Hemojuvelin protein from the overexpression lysate was verified.
References
Papanikolaou G, et al. (2004) Mutations in HFE2 cause iron overload in chromosome 1q-linked juvenile hemochromatosis. Nat Genet. 36(1):77-82.Babitt JL, et al. (2006) Bone morphogenetic protein signaling by hemojuvelin regulates hepcidin expression. Nat Genet. 38(5):531-9.Zhang AS, et al. (2008) Neogenin-mediated hemojuvelin shedding occurs after hemojuvelin traffics to the plasma membrane. J Biol Chem. 283(25):17494-502.

NCBI and Uniprot Product Information

NCBI GI #
NCBI GeneID
UniProt Accession #
Molecular Weight
426
NCBI Official Full Name
Hemojuvelin
NCBI Official Synonym Full Names
hemochromatosis type 2 (juvenile)
NCBI Official Symbol
HFE2
NCBI Official Synonym Symbols
JH; HJV; RGMC; HFE2A
NCBI Protein Information
hemojuvelin; haemojuvelin; RGM domain family member C; repulsive guidance molecule c; hemochromatosis type 2 protein
UniProt Protein Name
Hemojuvelin
Protein Family
UniProt Gene Name
HFE2
UniProt Synonym Gene Names
HJV; RGMC
UniProt Entry Name
RGMC_HUMAN

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. 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, Jul 2008]

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: extracellular space; cell surface; plasma membrane

Molecular Function: protein binding; coreceptor activity

Biological Process: BMP signaling pathway; axon guidance; iron ion homeostasis; positive regulation of transcription from RNA polymerase II promoter

Disease: Hemochromatosis, Type 2a

Research Articles on HJV

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Product Notes

The HJV hfe2 (Catalog #AAA8113830) is a Cell Lysate produced from Baculovirus-Insect cells and is intended for research purposes only. The product is available for immediate purchase. The immunogen sequence is 374aa. AAA Biotech's Hemojuvelin can be used in a range of immunoassay formats including, but not limited to, Western Blot (WB). WB: Use at an assay dependent dilution. 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. The amino acid sequence is listed below: Met1-Ser39 9. It is sometimes possible for the material contained within the vial of "Hemojuvelin, Cell Lysate" 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.

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