蛋白名称
Glucose-6-phosphate 1-dehydrogenase
免疫原
Purified recombinant fragment of human G6PD expressed in E. Coli.
特异性
G6PD Monoclonal Antibody detects endogenous levels of G6PD protein.
组成
Ascitic fluid containing 0.03% sodium azide,0.5% BSA, 50%glycerol.
稀释比例
WB: 1/500 - 1/2000. IHC: 1/200 - 1/1000. Flow cytometry: 1/200 - 1/400. ELISA: 1/10000.. IF 1:50-200
纯化工艺
Affinity purification
其他名称
G6PD; Glucose-6-phosphate 1-dehydrogenase; G6PD
背景
glucose-6-phosphate dehydrogenase(G6PD) Homo sapiens This gene encodes glucose-6-phosphate dehydrogenase. This protein is a cytosolic enzyme encoded by a housekeeping X-linked gene whose main function is to produce NADPH, a key electron donor in the defense against oxidizing agents and in reductive biosynthetic reactions. G6PD is remarkable for its genetic diversity. Many variants of G6PD, mostly produced from missense mutations, have been described with wide ranging levels of enzyme activity and associated clinical symptoms. G6PD deficiency may cause neonatal jaundice, acute hemolysis, or severe chronic non-spherocytic hemolytic anemia. Two transcript variants encoding different isoforms have been found for this gene. [provided by RefSeq, Jul 2008],
功能
catalytic activity:D-glucose 6-phosphate + NADP(+) = D-glucono-1,5-lactone 6-phosphate + NADPH.,disease:Defects in G6PD are the cause of chronic non-spherocytic hemolytic anemia (CNSHA) [MIM:305900]. Deficiency of G6PD is associated with hemolytic anemia in two different situations. First, in areas in which malaria has been endemic, G6PD-deficiency alleles have reached high frequencies (1% to 50%) and deficient individuals, though essentially asymptomatic in the steady state, have a high risk of acute hemolytic attacks. Secondly, sporadic cases of G6PD deficiency occur at a very low frequencies, and they usually present a more severe phenotype. Several types of CNSHA are recognized. Class-I variants are associated with severe NSHA; class-II have an activity <10% of normal; class-III have an activity of 10% to 60% of normal; class-IV have near normal activity.,function:Produces pentose su
