ⓘ Pyruvate dehydrogenase deficiency

                                     

ⓘ Pyruvate dehydrogenase deficiency

Pyruvate dehydrogenase deficiency is one of the most common neurodegenerative disorders associated with abnormal mitochondrial metabolism. PDCD is an X-linked disease that shows heterogeneous characteristics in both clinical presentation and biochemical abnormality. The pyruvate dehydrogenase complex is a multi-enzyme complex that plays a vital role as a key regulatory step in the central pathways of energy metabolism in the mitochondria.

                                     
  • Pyruvate dehydrogenase is an enzyme that catalyzes the reaction of pyruvate and a lipoamide to give the acetylated dihydrolipoamide and carbon dioxide
  • Pyruvate dehydrogenase complex PDC is a complex of three enzymes that converts pyruvate into acetyl - CoA by a process called pyruvate decarboxylation
  • Pyruvate dehydrogenase E1 component subunit alpha, somatic form, mitochondrial is an enzyme that in humans is encoded by the PDHA1 gene.The pyruvate dehydrogenase
  • Pyruvate dehydrogenase lipoamide beta, also known as pyruvate dehydrogenase E1 component subunit beta, mitochondrial or PDHE1 - B is an enzyme that in
  • Pyruvate dehydrogenase lipoamide alpha 2, also known as pyruvate dehydrogenase E1 component subunit alpha, testis - specific form, mitochondrial or PDHE1 - A
  • serves to reverse the effects of pyruvate dehydrogenase kinase upon pyruvate dehydrogenase Pyruvate dehydrogenase E1 is one of the three components
  • component of the pyruvate dehydrogenase complex found only in eukaryotes.Defects in this gene are a cause of pyruvate dehydrogenase deficiency which results
  • component of the multienzyme pyruvate dehydrogenase complex. The pyruvate dehydrogenase complex is responsible for the pyruvate decarboxylation step that
  • Lactate dehydrogenase LDH or LD is an enzyme found in nearly all living cells. LDH catalyzes the conversion of lactate to pyruvate and back, as it converts
  • Glucose - 6 - phosphate dehydrogenase deficiency G6PDD is an inborn error of metabolism that predisposes to red blood cell breakdown. Most of the time, those