Human Hemojuvelin/RGM-C/HJV ELISA Kit PicoKine®

Hemojuvelin (HJV/RGMc/HFE2) is a membrane-bound and soluble protein in mammals. It is a member of the repulsive guidance molecule family of proteins. In humans, the hemojuvelin protein is encoded by the HFE2 gene. 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.

Lots Lot 1 (pg/ml) Lot 2 (pg/ml) Lot 3 (pg/ml) Lot 4 (pg/ml) Mean (pg/ml) Standard Deviation CV (%) Sample 1 285 292 319 286 295 13.82 4.6% Sample 2 1139 1247 1212 1220 1204 39.97 3.3% Sample 3 5792 6174 5259 6098 5830 359.73 6.1% Lots Lot 1 (pg/ml) Lot 2 (pg/ml) Lot 3 (pg/ml) Lot 4 (pg/ml) Mean (pg/ml) Standard Deviation CV (%) Lots Lot 1 (pg/ml) Lot 2 (pg/ml) Lot 3 (pg/ml) Lot 4 (pg/ml) Mean (pg/ml) Standard Deviation CV (%) Sample 1 285 292 319 286 295 13.82 4.6% Sample 1 285 292 319 286 295 13.82 4.6% Sample 2 1139 1247 1212 1220 1204 39.97 3.3% Sample 2 1139 1247 1212 1220 1204 39.97 3.3% Sample 3 5792 6174 5259 6098 5830 359.73 6.1% Sample 3 5792 6174 5259 6098 5830 359.73 6.1%

Intra-Assay Precision Inter-Assay Precision Sample 1 2 3 1 2 3 n 16 16 16 24 24 24 Mean (pg/ml) 285 1139 5792 1123 1123 5755 Standard deviation 17.38 72.89 457.56 18.45 74.11 552.48 CV (%) 6.1% 6.4% 7.9% 6.6% 6.6% 9.6% Intra-Assay Precision Inter-Assay Precision Sample 1 2 3 1 2 3 n 16 16 16 24 24 24 Mean (pg/ml) 285 1139 5792 1123 1123 5755 Standard deviation 17.38 72.89 457.56 18.45 74.11 552.48 CV (%) 6.1% 6.4% 7.9% 6.6% 6.6% 9.6% Intra-Assay Precision Inter-Assay Precision Intra-Assay Precision Inter-Assay Precision Sample 1 2 3 1 2 3 Sample 1 2 3 1 2 3 n 16 16 16 24 24 24 n 16 16 16 24 24 24 Mean (pg/ml) 285 1139 5792 1123 1123 5755 Mean (pg/ml) 285 1139 5792 1123 1123 5755 Standard deviation 17.38 72.89 457.56 18.45 74.11 552.48 Standard deviation 17.38 72.89 457.56 18.45 74.11 552.48 CV (%) 6.1% 6.4% 7.9% 6.6% 6.6% 9.6% CV (%) 6.1% 6.4% 7.9% 6.6% 6.6% 9.6%

Acts as a bone morphogenetic protein (BMP) coreceptor. Through enhancement of BMP signaling regulates hepcidin (HAMP) expression and regulates iron homeostasis. .

Description|Quantity Pre-coated 96-well strip microplate|1 Standard|2 vials Biotinylated antibody (100x) |100ul Avidin-Biotin-Peroxidase Complex (100x) |100ul Sample Diluent|30ml Antibody Diluent|12ml Avidin-Biotin-Peroxidase Diluent|12ml Color Developing Reagent (TMB) |10ml Stop Solution|10ml Wash Buffer (25x) |20ml Adhesive plate sealers|4

Store at 4°C for 6 months, at -20°C for 12 months. Avoid multiple freeze-thaw cycles (Ships with gel ice, can store for up to 3 days in room temperature. Freeze upon receiving.)

Concentration (pg/ml) 0 156 312 625 1250 2500 5000 10000 O.D. 0.009 0.084 0.149 0.269 0.459 0.818 1.53 2.012 Concentration (pg/ml) 0 156 312 625 1250 2500 5000 10000 O.D. 0.009 0.084 0.149 0.269 0.459 0.818 1.53 2.012 Concentration (pg/ml) 0 156 312 625 1250 2500 5000 10000 Concentration (pg/ml) 0 156 312 625 1250 2500 5000 10000 O.D. 0.009 0.084 0.149 0.269 0.459 0.818 1.53 2.012 O.D. 0.009 0.084 0.149 0.269 0.459 0.818 1.53 2.012

Microplate Reader capable of reading absorbance at 450nm. Incubator. Automated plate washer (optional) . Pipettes and pipette tips capable of precisely dispensing 0.5 μl through 1 ml volumes of aqueous solutions. Multichannel pipettes are recommended for large amount of samples. Deionized or distilled water. 500ml graduated cylinders. Test tubes for dilution.