Review of the pathogenetic problem of hereditary ataxia
Gurgone, G.; Gaglio, R.M.; Piccoli, F.
Rivista di Neurologia 54(6): 419-433
1984
ISSN/ISBN: 0035-6344 PMID: 6396827 Document Number: 226711
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.