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Regulation of pyruvate dehydrogenase (PDH) complex

Summary

Organism

Homo sapiens (human)

Reactome

R-HSA-204174

PubChem

R-HSA-204174

Description

The mitochondrial pyruvate dehydrogenase (PDH) complex catalyzes the oxidative decarboxylation of pyruvate, linking glycolysis to the tricarboxylic acid cycle and fatty acid synthesis. PDH inactivation is crucial for glucose conservation when glucose is scarce, while adequate PDH activity is required to allow both ATP and fatty acid production from glucose. The mechanisms that control human PDH activity include its phosphorylation (inactivation) by pyruvate dehydrogenase kinases (PDK 1-4) and its dephosphorylation (activation, reactivation) by pyruvate dehydrogenase phosphate phosphatases (PDP 1 and 2). Isoform-specific differences in kinetic parameters, regulation, and phosphorylation site specificity of the PDKs introduce variations in the regulation of PDC activity in differing endocrine and metabolic states (Sugden and Holness 2003). Further, PDH is inhibited by SIRT4 and the drug dichloroacetic acid (DCA).

Click on a node on the pathway to see its details. Glycoproteins are marked with a glycoprotein icon in their name.

SPV: a JavaScript Signaling Pathway Visualizer, Bioinformatics, 2018

Displaying **all 8** entries
UniProt ID	Protein Name	Gene Symbol	Pathway Viewer
O00330	Pyruvate dehydrogenase protein X component, mitochondrial	PDHX PDX1	view
P08559	Pyruvate dehydrogenase E1 component subunit alpha, somatic form, mitochondrial	PDHA1 PHE1A	view
P09622	Dihydrolipoyl dehydrogenase, mitochondrial	DLD GCSL LAD PHE3	view
P10515	Dihydrolipoyllysine-residue acetyltransferase component of pyruvate dehydrogenase complex, mitochondrial	DLAT DLTA	view
P11177	Pyruvate dehydrogenase E1 component subunit beta, mitochondrial	PDHB PHE1B	view
Q15118	[Pyruvate dehydrogenase (acetyl-transferring)] kinase isozyme 1, mitochondrial	PDHK1 PDK1	view
Q15120	[Pyruvate dehydrogenase (acetyl-transferring)] kinase isozyme 3, mitochondrial	PDHK3 PDK3	view
Q9P0J1	[Pyruvate dehydrogenase [acetyl-transferring]]-phosphatase 1, mitochondrial	PDP PDP1 PPM2C	view

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