Metabolism of Amino Acids and Organic Acids

  • Kay Tanaka
  • Leon E. Rosenberg


To those interested in mammalian biochemistry and metabolism, it has become axiomatic that pyruvate is a key intermediate in such major pathways as those concerned with anaerobic glycolysis, gluconeogenesis, and alanine formation. Until recently, there was a widespread belief among clinical investigators that inborn errors of pyruvate metabolism would not be found. This skepticism had two foundations: First, it was argued, serious defects in utilization of such a key intermediate would be incompatible with successful embryonic or fetal development, and thus affected humans would not survive the gestational interval. Second, since it has been known for some time that pyruvate metabolism is under major regulation by other events such as the redox state of the cell, it was held that the accumulation of pyruvate observed under diverse circumstances would always be secondary to some major tissue insult, rather than being a reflection of a primary, inborn enzymatic disturbance in pyruvate formation or disposition.


Lactic Acidosis Pyruvate Kinase Pyruvate Carboxylase Pyruvate Dehydrogenase Complex Pyruvate Decarboxylase 


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Copyright information

© Plenum Publishing Corporation 1979

Authors and Affiliations

  • Kay Tanaka
    • 1
  • Leon E. Rosenberg
    • 1
  1. 1.Department of Human GeneticsYale University School of MedicineNew HavenUSA

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