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[edit | edit source]
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[edit | edit source]
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[edit | edit source]
Mutations in the GPD1 gene have been associated with transient infantile hypertriglyceridemia, a rare metabolic disorder characterized by severe hypertriglyceridemia during infancy.
See also[edit | edit source]
- Glycerol-3-phosphate dehydrogenase
- Glycerol phosphate shuttle
- Dihydroxyacetone phosphate
- Lipid metabolism
References[edit | edit source]
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Credits:Most images are courtesy of Wikimedia commons, and templates Wikipedia, licensed under CC BY SA or similar.Contributors: Prab R. Tumpati, MD