PDHB

The pyruvate dehydrogenase (PDH) complex is a nuclear-encoded mitochondrial multienzyme complex that catalyzes the overall conversion of pyruvate to acetyl-CoA and carbon dioxide, and provides the primary link between glycolysis and the tricarboxylic acid (TCA) cycle. The PDH complex is composed of multiple copies of three enzymatic components: pyruvate dehydrogenase (E1), dihydrolipoamide acetyltransferase (E2) and lipoamide dehydrogenase (E3). The E1 enzyme is a heterotetramer of two alpha and two beta subunits. This gene encodes the E1 beta subunit. Mutations in this gene are associated with pyruvate dehydrogenase E1-beta deficiency.

pyruvate dehydrogenase (lipoamide) beta

The pyruvate dehydrogenase complex catalyzes the overall conversion of pyruvate to acetyl-CoA and CO2, and thereby links the glycolytic pathway to the tricarboxylic cycle.

Acetyl-CoA biosynthetic process from pyruvateManual Assertion Based On ExperimentIDA:UniProtKB
Glucose metabolic processIEA:UniProtKB-KW
Mitochondrial acetyl-CoA biosynthetic process from pyruvate1 PublicationIC:MGI
Tricarboxylic acid cycleManual Assertion Based On ExperimentTAS:ProtInc

Mitochondrion matrix

Pyruvate dehydrogenase E1-beta deficiency (PDHBD):
An enzymatic defect causing primary lactic acidosis in children. It is associated with a broad clinical spectrum ranging from fatal lactic acidosis in the newborn to chronic neurologic dysfunction with structural abnormalities in the central nervous system without systemic acidosis.