蛋白名称
G6PD(Glucose 6 Phosphate Dehydrogenase)
组成
PBS, 50% glycerol, 0.05% Proclin 300, 0.05%BSA
来源
Monoclonal, Rabbit,IgG
稀释比例
IHC 1:200-1:1000; WB 1:2000-1:10000; IF 1:200-1:1000; ELISA 1:5000-1:20000; IP 1:50-1:200; Note: For IHC, we suggest antigen retrieval with TE buffer pH 9.0
储存
-15°C to -25°C/1 year(Do not lower than -25°C)
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 sugars for nucleic acid synthesis and main producer of NADPH reducing power.,miscellaneous:Has NADP both as cofactor (bound to the N-terminal domain) and as structural element bound to the C-terminal domain.,online information:G6PD deficiency resource,online information:G6PD mutation database,online information:The Singapore human mutation and polymorphism database,pathway:Carbohydrate degradation; pentose phosphate pathway.,pathway:Carbohydrate degradation; pentose phosphate pathway; D-ribulose 5-phosphate from D-glucose 6-phosphate (oxidative stage): step 1/3.,polymorphism:The sequence shown is that of variant B, the most common variant.,similarity:Belongs to the glucose-6-phosphate dehydrogenase family.,subunit:Homodimer or homotetramer.,tissue specificity:The long isoform is found in lymphoblasts, granulocytes and sperm.,
