The spectrum of pyruvate dehydrogenase complex
deficiency: clinical, biochemical and genetic features in 371 patients.
Evidence for existence of tissue-specific regulation of the mammalian pyruvate dehydrogenase complex
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Abbreviations are as follows: adenomatous polyposis coli (APC); alpha ceto-glutarate (a-KG); beta-catenin proteasomal degradation: CPD; catenin binding domain (CBD); Dishevelled (Dsh); Frizzled (Fzd); glycogen synthase kinase-3beta (GSK-3beta); lactate dehydrogenase (LDH); low-density lipoprotein receptor-related protein 5/6 (LRP5/6); monocarboxylate lactate transporter-1 (MCT-1); peroxisome proliferator-activated receptor gamma (PPAR gamma); pyruvate dehydrogenase complex
(PDH); pyruvate dehydrogenase kinase (PDK); T-cell factor/lymphoid enhancer factor (TCF/LEF); tricarboxylic acid (TCA); *: Wnt targets: PDK, cMyc, MCT-1, and cyclin D1.
The oxidation of coenzymes (reduced in glycolysis, reaction catalysed by pyruvate dehydrogenase complex
, [beta] oxidation, and Krebs cycle) in the mitochondrial respiratory chain and the consequent mitochondrial oxidative phosphorylation represent the major pathway of intracellular energy production in the form of ATP for all mammalian cells, except for erythrocytes.
In terms of glucose combustion, NADH is mainly generated by the glycolytic pathway, by pyruvate dehydrogenase complex
via dihydrolipoamide dehydrogenase , and by the TCA cycle .