Recombinant Human GDH/6PGL endoplasmic bifunctional protein (H6PD)

Uniprot Id

O95479

Target Species

Human

Target Name

H6PD

Target Full Name

GDH/6PGL endoplasmic bifunctional protein

Target Function

Bifunctional enzyme localized in the lumen of the endoplasmic reticulum that catalyzes the first two steps of the oxidative branch of the pentose phosphate pathway/shunt, an alternative to glycolysis and a major source of reducing power and metabolic intermediates for biosynthetic processes. Has a hexose-6-phosphate dehydrogenase activity, with broad substrate specificity compared to glucose-6-phosphate 1-dehydrogenase/G6PD, and catalyzes the first step of the pentose phosphate pathway. In addition, acts as a 6-phosphogluconolactonase and catalyzes the second step of the pentose phosphate pathway. May have a dehydrogenase activity for alternative substrates including glucosamine 6-phosphate and glucose 6-sulfate. The main function of this enzyme is to provide reducing equivalents such as NADPH to maintain the adequate levels of reductive cofactors in the oxidizing environment of the endoplasmic reticulum. By producing NADPH that is needed by reductases of the lumen of the endoplasmic reticulum like corticosteroid 11-beta-dehydrogenase isozyme 1/HSD11B1, indirectly regulates their activity.

Target Involvement

Cortisone reductase deficiency 1 (CORTRD1)

Target Subcellular Location

Endoplasmic reticulum lumen.

Target Protein Families

Glucose-6-phosphate dehydrogenase family; Glucosamine/galactosamine-6-phosphate isomerase family, 6-phosphogluconolactonase subfamily

Target Tissue Specificity

Present in most tissues examined, strongest in liver.

Target Synonyms

6 phosphogluconolactonase; 6-phosphogluconolactonase; 6PGL; CORTRD1; G6PD; H form; G6PDH; G6PE_HUMAN; GDH; GDH/6PGL endoplasmic bifunctional protein; Glucose 1 dehydrogenase; Glucose 6 phosphate dehydrogenase; salivary; Glucose dehydrogenase; Glucose dehyrogenase; Gpd1; H6pd; Hexose 6 phosphate dehydrogenase (glucose 1 dehydrogenase); Hexose 6 phosphate dehydrogenase; Hexose-6-phosphate dehydrogenase

Target Background

There are 2 forms of glucose-6-phosphate dehydrogenase. G form is X-linked and H form, encoded by this gene, is autosomally linked. This H form shows activity with other hexose-6-phosphates, especially galactose-6-phosphate, whereas the G form is specific for glucose-6-phosphate. Both forms are present in most tissues, but H form is not found in red cells.