GDH/6PGL endoplasmic bifunctional protein; endoplasmic bifunctional protein precursor [Includes: glucose 1-dehydrogenase, hexose-6-phosphate dehydrogenase, 6-phosphogluconolactonase] (6PGL, H6PD, G1DH)
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Function
- oxidizes glucose-6-phosphate & glucose, as well as other hexose-6-phosphates
beta-D-glucose + NAD+/NADP+ <--> D-glucono-1,5-lactone + NADH/NADPH
D-glucose 6-phosphate + NAD+/NADP+ <--> D-glucono-1,5-lactone 6-phosphate + NADH/NADPH
6-phospho-D-glucono-1,5-lactone + H2O <--> 6-phospho-D-gluconate
Structure
- in the N-terminal section; belongs to the glucose-6-phosphate dehydrogenase family
- in the C-terminal section; belongs to the glucosamine/galactosamine-6-phosphate isomerase family, 6-phosphogluconolactonase subfamily
Compartment
Expression
- present in most tissues
- strongest expression in liver
Pathology
- defects in H6PD are a cause of cortisone reductase deficiency
More general terms
Additional terms
References
- ↑ UniProt http://www.uniprot.org/uniprot/O95479.html
- ↑ SHMPD; The Singapore human mutation and polymorphism database http://shmpd.bii.a-star.edu.sg/gene.php?genestart=A&genename=H6PD