[Review of the pathogenetic problem of hereditary ataxia]

Riv Neurol. 1984 Nov-Dec;54(6):419-33.
[Article in Italian]

Abstract

In the inherited ataxias, a heterogeneous group of relatively rare and progressive neurological disorders, abnormalities in pyruvate metabolism have been described. Pyruvate is involved in the glycolitic pathway as an important step, and utilized in the Krebs cycle, the main energy source in the brain. Furthermore, pyruvate and other intermediates in the Krebs cycle, can also serve as a precursors of amino acids for which a role as a neurotransmitter has been shown. The explanation of the basic biochemistry may serve as a basis for a more steady knowledge of the clinical and pathological findings of such diseases.

Publication types

  • English Abstract
  • Review

MeSH terms

  • Amino Acids / metabolism
  • Ataxia / genetics*
  • Ataxia / metabolism
  • Cerebellar Ataxia / genetics
  • Cerebellar Ataxia / metabolism
  • Citric Acid Cycle
  • Diabetes Complications
  • Friedreich Ataxia / metabolism
  • Glycolysis
  • Humans
  • Oxidoreductases / metabolism
  • Pyruvate Dehydrogenase Complex / metabolism
  • Pyruvates / metabolism
  • Pyruvic Acid
  • gamma-Aminobutyric Acid / metabolism

Substances

  • Amino Acids
  • Pyruvate Dehydrogenase Complex
  • Pyruvates
  • gamma-Aminobutyric Acid
  • Pyruvic Acid
  • Oxidoreductases