Outcome of pyruvate dehydrogenase
deficiency treated with ketogenic diets.
This stimulates anaerobic glycolysis, inhibits pyruvate dehydrogenase
, inhibits mitochondrial oxidative phosphorylation, stimulates heme oxygenase, stimulates VEGF and activates nitric oxide synthase.
KEY WORDS: cancer, dichloroacetate, glutathione transferase zeta, hereditary tyrosinemia, maleylacetoacetate isomerase, mitochondrial disease, peripheral neuropathy, pyruvate dehydrogenase
This lead to the successful testing of a small molecule inhibitor of pyruvate dehydrogenase
kinase in animal tumors, and we now have approval to begin a clinical trial in patients with solid tumors.
Mutations in the X-linked pyruvate dehydrogenase
(E1) [alpha] subunit gene (PDHA1) in patients with a pyruvate dehydrogenase
Some of the acetyl-CoA groups formed in mitochondria by pyruvate dehydrogenase
(PDH;  EC 1.
Redox stress modulated enzymes include pyruvate dehydrogenase
, nitric oxide synthase, cystathione beta synthase and hemeoxygenase.
The pyruvate dehydrogenase
complex (PDHc)  plays a pivotal role in energy metabolism: it catalyzes the oxidative decarboxylation of pyruvate to acetyl-CoA, which is an important entry point for activated two-carbon acetyl units into the tricarboxylic acid cycle.
Antibody binding and inhibition of pyruvate dehydrogenase
(PDH) in sera from patients with primary biliary cirrhosis.
Lactate concentrations in CSF have also been reported to be increased in individuals with several inborn errors of metabolism (IEM) that can affect the central nervous system, such as pyruvate dehydrogenase
(PDH) deficiency (5), and respiratory chain (RC) disorders (6).
The major mitochondrial antigen was identified as the 74-kDa E2 subunit of the pyruvate dehydrogenase
complex (PDC), a member of the 2-oxoacid dehydrogenase complex family (4).
This last group includes disorders of gluconeogenesis, the pyruvate dehydrogenase
complex, the Krebs cycle, and the mitochondrial electron transport chain .