bs-9519R

DATASHEET

Host: Rabbit

Target Protein: BPGM

Immunogen Range: 101-200/259

Clonality: Polyclonal

Isotype: IgG

Entrez Gene: 669

Source: KLH conjugated synthetic peptide derived from human BPGM

Purification: Purified by Protein A.

Storage Buffer: 0.01M TBS(pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.

Storage: Shipped at 4°C. Store at -20°C for one year. Avoid repeated freeze/thaw cycles.

Background:

BPGM (2,3-bisphosphoglycerate mutase) is a 259 amino acid protein that belongs to the phosphoglycerate mutase family and exists as a homodimer that plays a crucial role in the regulation of hemoglobin oxygen. Specifically, BPGM catalyzes the conversion of 3-D-glyceroyl phosphate to 2,3-bisD-glycerate (2,3-BPG), a reaction that is essential for controlling the concentration of 2,3-BPG within the cell. The gene encoding BPGM maps to human chromosome 7, which houses over 1,000 genes and comprises nearly 5% of the human genome. Defects in some of the genes localized to chromosome 7 have been linked to Osteogenesis imperfecta, Williams-Beuren syndrome, Pendred syndrome, Lissencephaly, Citrullinemia and Shwachman-Diamond syndrome. Involvement in disease:Defects in BPGM are the cause of bisphosphoglycerate mutase deficiency (BPGMD) . A disease characterized by hemolytic anemia, splenomegaly, cholelithiasis and cholecystitis.

Size: 100ul

Concentration: 1ug/ul

Applications: WB(1:300-5000)
FCM(1:20-100)
IHC-P(1:200-400)
IF(IHC-P)(1:50-200)

Cross Reactive Species: Human
Mouse
Rat

For research use only. Not intended for diagnostic or therapeutic use.