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glucose-6-phosphate 1-dehydrogenase (G6PD)

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: - glucose-6-phosphate dehydrogenase in specimen

Interactions

molecular events

Related

G6PD gene mutation G6PD genotyping 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

General

carbohydrate dehydrogenase oligomerizing protein phosphoprotein

Properties

SIZE: entity length = 515 aa MW = 59 kD MOTIF: cofactor-binding site [39-45] COFACTOR-BOUND: NADP MOTIF: cofactor-binding site [40-40] COFACTOR-BOUND: NADP cofactor-binding site [72-72] COFACTOR-BOUND: NADP binding site SITE: 201-201 FOR-BINDING-OF: Substrate binding site SITE: 205-205 FOR-BINDING-OF: Substrate cofactor-binding site [238-238] COFACTOR-BOUND: NADP histidine residue {H263} cofactor-binding site [357-357] COFACTOR-BOUND: NADP Tyr phosphorylation site {Y401} cofactor-binding site [487-487] COFACTOR-BOUND: NADP Tyr phosphorylation site {Y507}

Database Correlations

OMIM 305900 MORBIDMAP 305900 UniProt P11413 PFAM correlations Kegg hsa:2539 ENZYME 1.1.1.49

References

  1. UniProt :accession P11413
  2. G6PD; G6PD deficiency resource http://rialto.com/g6pd/
  3. G6PDdb; G6PD mutation database http://www.rubic.rdg.ac.uk/g6pd/
  4. GeneReviews https://www.genecards.org/cgi-bin/carddisp.pl?gene=G6PD
  5. SHMPD; The Singapore human mutation and polymorphism database http://shmpd.bii.a-star.edu.sg/gene.php?genestart=A&genename=G6PD