glucose-6-phosphate 1-dehydrogenase (G6PD)
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Function
- catalyzes 1st step in hexose monophosphate shunt
- generates NADPH from NADP+ for cytoplasmic reducing potential
- main producer of NADPH reducing power
- produces pentose sugars for nucleic acid synthesis
- carbohydrate degradation; pentose phosphate pathway
- D-ribulose 5-phosphate from D-glucose 6-phosphate (oxidative stage): step 1/3
D-glucose 6-phosphate + NADP+
<-->
D-glucono-1,5-lactone 6-phosphate + NADPH
Structure
- homodimer or homotetramer
- belongs to the glucose-6-phosphate dehydrogenase family
- NADP is bound both as cofactor (N-teminal) & as structural element (C-terminal)
Alternative splicing
named isoforms=2
- short isoform erythrocytes
- long isoform lymphoblasts, granulcytes, sperm
Expression
- long isoform is found in lymphoblasts, granulocytes & sperm
Pathology
- deficiency relatively common (see G6PD deficiency)
Polymorphism
- B variant most common
- A variant associated with less severe hemolysis than complete deficiency
Laboratory
More general terms
Additional terms
- glucose-6-phosphate dehydrogenase (G6PD) in erythrocytes
- glucose-6-phosphate dehydrogenase [G6PD] deficiency; chronic non-spherocytic hemolytic anemia (CNSHA)
- hexose monophosphate shunt, pentose phosphate pathway or phosphogluconate shunt
References
- ↑ UniProt http://www.uniprot.org/uniprot/P11413.html
- ↑ G6PD; G6PD deficiency resource http://rialto.com/g6pd/
- ↑ G6PDdb; G6PD mutation database http://www.rubic.rdg.ac.uk/g6pd/
- ↑ GeneReviews https://www.genecards.org/cgi-bin/carddisp.pl?gene=G6PD
- ↑ SHMPD; The Singapore human mutation and polymorphism database http://shmpd.bii.a-star.edu.sg/gene.php?genestart=A&genename=G6PD