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Rabbit  Anti-BPGM/FITC
Cat. Number:
bs-9519R-FITC
Quantity size:
100ul
Concentration:
1mg/ml  Buffer = 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
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-phospho-D-glyceroyl phosphate to 2,3-bisphospho-D-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.
Also known as:
2,3-bisphosphoglycerate mutase; 2,3-bisphosphoglycerate synthase; 3-bisphosphoglycerate mutase; 3-bisphosphoglycerate synthase; 3-diphosphoglycerate mutase; Ab2 098; AI323730; AL022789; Bisphosphoglycerate mutase; BPG dependent PGAM; BPG-dependent PGAM ; Bpgm; BPGM; C86192; DPGM; Erythrocyte 2,3 bisphosphoglycerate mutase; PMGE_HUMAN; 2,3-bisphosphoglycerate mutase, erythrocyte; erythrocyte; 2 antibody.
Specificity:
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Rabbit Polyclonal IgG, affinity purified by Protein A.
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Reacts with: (predicted: )
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Immunogen: KLH conjugated synthetic peptide derived from human BPGM.
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Predicted Molecular Weight: 30kDa.
Storage:
0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol. Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. The lyophilized antibody is stable at room temperature for at least one month and for greater than a year when kept at -20°C. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 °C.
Application:

Excitation spectrum: 495nm
Emission spectrum: 519nm
Not yet tested in other applications.
Optimal working dilutions must be determined by the end user.
Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.