红细胞2,3 - 二磷酸甘油酸合成酶抗体

红细胞2,3 - 二磷酸甘油酸合成酶抗体Quantity size:

0.2ml

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.红细胞2,3 - 二磷酸甘油酸合成酶抗体 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,红细胞2,3 - 二磷酸甘油酸合成酶抗体 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; 红细胞2,3 - 二磷酸甘油酸合成酶抗体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: Human, Mouse, Rat, Cow, Rabbit, Sheep, .

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Immunogen: KLH conjugated synthetic peptide derived from human BPGM.

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红细胞2,3 - 二磷酸甘油酸合成酶抗体Predicted Molecular Weight: 30kDa.

Storage:

Shipped at 4℃, Store at -20℃ (Avoid repeated freeze/thaw cycles).

Application:

WB=1:100-500  ELISA=1:500-1000  IHC-P=1:100-500  IHC-F=1:100-500  IF=1:50-200   
Not yet tested in other applications. 
Optimal working dilutions must be determined by the end user.

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