Abstract
Relative carnitine deficiency is important in the pathophysiology of several disorders, including Reye’s syndrome and organic acidemias. In acute clinical crises, carnitine serves as a “buffer,” trapping toxic acyl compounds. Mitochondrial failure develops in carnitine deficiency when there is insufficient tissue carnitine available to buffer toxic acyl-CoA metabolites. Toxic levels of acyl-Co A impair the citrate cycle, gluconeogenesis, the urea cycle, and fatty-acid oxidation. Carnitine replacement therapy is safe and induces excretion of toxic acyl groups in the urine.
Original language | English (US) |
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Pages (from-to) | 1041-1045 |
Number of pages | 5 |
Journal | Neurology |
Volume | 35 |
Issue number | 7 |
DOIs | |
State | Published - Jul 1985 |
ASJC Scopus subject areas
- Clinical Neurology