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We have identified a p.R158H PDK3 mutation in a Korean X-linked dominant Charcot-Marie-Tooth type 6 family
Accordingly, elevated levels of PDK1, PDK3, and PKM2 and reduced PK activity could be observed in iPSCs and human embryonic stem cells in the undifferentiated state
Reduced pyruvate flux due to R158H mutant PDK3-mediated hyper-phosphorylation of the pyruvate dehydrogenase (show PDP Proteins) complex is the underlying pathogenic cause of peripheral neuropathy in X-linked dominant Charcot-Marie-Tooth disease.
Findings suggest the HIF-1 (show HIF1A Proteins)/PDK3 bioenergetic pathway as a new target for therapeutic intervention in metastatic melanoma.
analysis of residues in L2 and residues in the C-terminal region and the lipoyl-binding pocket of PDK3 which are critical determinants for the cross-talk between L2 and PDK3, which up-regulates PDK3 activity
PDK2 (show PDK2 Proteins), PDK3 and PDK4 (show PDK4 Proteins) are primary PPARbeta (show PPARD Proteins)/delta target genes in humans underlining the importance of the receptor in the control of metabolism
Distinct structural mechanisms for inhibition of PDK3 by AZD7545, dichloroacetate, and radicol.
increased PDK3 expression due to elevated HIF-1alpha (show HIF1A Proteins) in cancer cells may play critical roles in metabolic switch during cancer progression and chemoresistance in cancer therapy
The pyruvate dehydrogenase (PDH) complex is a nuclear-encoded mitochondrial multienzyme complex that catalyzes the overall conversion of pyruvate to acetyl-CoA and CO(2). It provides the primary link between glycolysis and the tricarboxylic acid (TCA) cycle, and thus is one of the major enzymes responsible for the regulation of glucose metabolism. The enzymatic activity of PDH is regulated by a phosphorylation/dephosphorylation cycle, and phosphorylation results in inactivation of PDH. The protein encoded by this gene is one of the three pyruvate dehydrogenase kinases that inhibits the PDH complex by phosphorylation of the E1 alpha subunit. This gene is predominantly expressed in the heart and skeletal muscles. Alternatively spliced transcript variants encoding different isoforms have been found for this gene.
pyruvate dehydrogenase kinase, isozyme 3
, pyruvate dehydrogenase kinase, isoenzyme 3
, pyruvate dehydrogenase kinase 3
, pyruvate dehydrogenase [lipoamide] kinase isozyme 3, mitochondrial-like
, [Pyruvate dehydrogenase [lipoamide]] kinase isozyme 3, mitochondrial
, pyruvate dehydrogenase, lipoamide, kinase isozyme 3, mitochondrial
, Pyruvate dehydrogenase kinase 3