Glycerol-3-phosphate dehydrogenase 1

Glycerol-3-phosphate dehydrogenase 1 (GPD1) is an enzyme that in humans is encoded by the GPD1 gene. This enzyme is part of the glycerol phosphate shuttle, a metabolic pathway involved in the transfer of reducing equivalents from the cytosol to the mitochondria.

Function

GPD1 catalyzes the reversible redox conversion of dihydroxyacetone phosphate (also known as glycerone phosphate) to sn-glycerol 3-phosphate. This reaction is an important step in the glycolysis pathway and the lipid metabolism pathway, as it links carbohydrate and lipid metabolism.

Structure

The GPD1 enzyme is a dimer composed of two identical subunits. Each subunit contains a NAD+/NADH binding domain and a substrate-binding domain. The enzyme's active site, where the redox reaction takes place, is located at the interface between the two domains.

Clinical significance

Mutations in the GPD1 gene have been associated with transient infantile hypertriglyceridemia, a rare metabolic disorder characterized by severe hypertriglyceridemia during infancy.

See also

• Glycerol-3-phosphate dehydrogenase
• Glycerol phosphate shuttle
• Dihydroxyacetone phosphate
• Lipid metabolism

Glucuronate metabolism

Pentose interconversion

Inositol metabolism

Cellulose and sucrose
metabolism

Starch and glycogen
metabolism

Other sugar
metabolism

Pentose phosphate pathway

Glycolysis and Gluconeogenesis

Amino sugars metabolism

Small amino acid synthesis

Branched amino acid
synthesis

Purine biosynthesis

Histidine metabolism

Aromatic amino
acid synthesis

Pyruvate
decarboxylation

Fermentation

Fatty acid
metabolism

Urea cycle

Aspartate amino acid
group synthesis

Porphyrins and
corrinoids
metabolism

Citric acid cycle

Glutamate amino
acid group
synthesis

Pyrimidine biosynthesis

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