catalog number :
MBS701344
products type :
ELISA Kit
products full name :
human hemochromatosis type 2/uvenile-uman homolog, HFE2 LISA Kit ELISA Kit
products short name :
hemochromatosis type 2 (juvenile)
products name syn :
human hemochromatosis type 2/uvenile-uman homolog; HFE2 LISA Kit; HFE2A; HJV; JH; MGC23953; RGMC; RGM domain family; member C; haemojuvelin; hemochromatosis type 2 protein; hemojuvelin; hemochromatosis type 2 (juvenile)
other names :
hemojuvelin isoform b; 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 :
HFE2
other gene names :
HFE2; HFE2; JH; HJV; RGMC; HFE2A; HJV; RGMC
uniprot entry name :
RGMC_HUMAN
specificity :
This assay has high sensitivity and excellent specificity for detection of Human HFE2. No significant cross-reactivity or interference between Human HFE2 and analogues was observed.
storage stability :
Unopened test kits should be stored at 2 to 8 degree C upon receipt. Please refer to pdf manual for further storage instructions.
other info1 :
Samples: Serum, plasma, tissue homogenates. Detection Range: 125 ng/ml-4000 ng/ml. Sensitivity: 62.5 ng/ml
other info2 :
Intra-assay Precision: Intra-assay Precision (Precision within an assay): CV% is less than 8%. Three samples of known concentration were tested twenty times on one plate to assess. Inter-assay Precision (Precision between assays): CV% is less than 10%. Three samples of known concentration were tested in twenty assays to assess. Detection Wavelength: 450 nm. Sample Volume: 50-100ul
products description :
Principle of the Assay: The microtiter plate provided in this kit has been pre-coated with an antibody specific to HFE2. Standards or samples are then added to the appropriate microtiter plate wells with a Horseradish Peroxidase (HRP) -conjugated antibody preparation specific for HFE2 and incubated. Then substrate solutions are added to each well. The enzyme-substrate reaction is terminated by the addition of a sulphuric acid solution and the color change is measured spectrophotometrically at a wavelength of 450 nm +/- 2 nm. The concentration of HFE2 in the samples is then determined by comparing the O.D. of the samples to the standard curve.
ncbi acc num :
NP_660320.3
ncbi gb acc num :
NM_145277.4
ncbi mol weight :
45,080 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 :
Function: 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.
size4 :
10x96-Strip-Wells