Search
GDH/6PGL endoplasmic bifunctional protein; endoplasmic bifunctional protein precursor [Includes: glucose 1-dehydrogenase, hexose-6-phosphate dehydrogenase, 6-phosphogluconolactonase] (6PGL, H6PD, G1DH)
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:
- endoplasmic reticulum lumen, microsomes
Expression:
- present in most tissues
- strongest expression in liver
Pathology:
- defects in H6PD are a cause of cortisone reductase deficiency
Related
6-phosphogluconolactonase (6PGL, PGLS)
General
dehydrogenase
glycoprotein
human longevity protein
Properties
SIZE: entity length = 791 aa
MW = 89 kD
COMPARTMENT: endoplasmic reticulum
MOTIF: signal sequence {1-19}
Glucose 1-dehydrogenase {20-526}
MOTIF: cofactor-binding site [34-34]
COFACTOR-BOUND: NADP
cofactor-binding site [66-66]
COFACTOR-BOUND: NADP
N-glycosylation site {N157}
binding site
SITE: 204-204
FOR-BINDING-OF: Substrate
binding site
SITE: 208-208
FOR-BINDING-OF: Substrate
histidine residue {H267}
N-glycosylation site {N282}
Linker {527-540}
6-phosphogluconolactonase {541-791}
MOTIF: N-glycosylation site {N683}
Database Correlations
OMIM correlations
UniProt O95479
PFAM correlations
Entrez Gene 9563
Kegg hsa:9563
ENZYME correlations
References
- UniProt :accession O95479
- SHMPD; The Singapore human mutation and polymorphism database
http://shmpd.bii.a-star.edu.sg/gene.php?genestart=A&genename=H6PD