Mouse Hemojuvelin ELISA Kit | MyBioSource MBS728146 product information

product summary

company name :

MyBioSource

product type :

ELISA/assay

product name :

Mouse Hemojuvelin ELISA Kit

catalog :

MBS728146

quantity :

48-Strip-Wells

price :

470 USD

more info or order :

MyBioSource product webpage

product information

catalog number :

MBS728146

products type :

ELISA Kit

products full name :

Mouse Hemojuvelin ELISA Kit

products short name :

Hemojuvelin

other names :

hemojuvelin isoform a; Hemojuvelin; hemojuvelin; haemojuvelin; RGM domain family member C; repulsive guidance molecule c; hemochromatosis type 2 protein; hemochromatosis type 2 (juvenile); Hemochromatosis type 2 protein; RGM domain family member C

products gene name :

HJV

other gene names :

HFE2; HFE2; JH; HJV; RGMC; HFE2A; HJV; RGMC

uniprot entry name :

RGMC_HUMAN

reactivity :

Mouse

storage stability :

Store all reagents at 2-8 degree C.

other info1 :

Samples: Serum, plasma, Cell Culture Supernatants, body fluid and tissue homogenate. Assay Type: Sandwich. Sensitivity: 1.0 pg/mL.

other info2 :

Intended Uses: This HJV ELISA kit is a 1.5 hour solid-phase ELISA designed for the quantitative determination of Mouse HJV. This ELISA kit for research use only, not for therapeutic or diagnostic applications!

products categories :

Cardiovascular

products description :

Principle of the Assay: HJV ELISA kit applies the quantitative sandwich enzyme immunoassay technique. The microtiter plate has been pre-coated with a monoclonal antibody specific for HJV. Standards or samples are then added to the microtiter plate wells and HJV if present, will bind to the antibody pre-coated wells. In order to quantitatively determine the amount of HJV present in the sample, a standardized preparation of horseradish peroxidase (HRP) -conjugated polyclonal antibody, specific for HJV are added to each well to "sandwich" the HJV immobilized on the plate. The microtiter plate undergoes incubation, and then the wells are thoroughly washed to remove all unbound components. Next, substrate solutions are added to each well. The enzyme (HRP) and substrate are allowed to react over a short incubation period. Only those wells that contain HJV and enzyme-conjugated antibody will exhibit a change in color. The enzyme-substrate reaction is terminated by addition of a sulphuric acid solution and the color change is measured spectrophotometrically at a wavelength of 450 nm. A standard curve is plotted relating the intensity of the color (O.D.) to the concentration of standards. The HJV concentration in each sample is interpolated from this standard curve.

ncbi gi num :

47458048

ncbi acc num :

NP_998818.1

ncbi gb acc num :

NM_213653.3

ncbi mol weight :

21,463 Da

ncbi pathways :

Axon Guidance Pathway (105688); BMP Receptor Signaling Pathway (137948); Developmental Biology Pathway (477129); Netrin-1 Signaling Pathway (160999)

ncbi summary :

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 summary :

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

size1 :

48-Strip-Wells

price1 :

470 USD

size2 :

96-Strip-Wells

price2 :

675

more info or order :

MyBioSource product webpage