Carnitine deficiency, organic acidemias, and reye’s syndrome

David A. Stumpf*, W. Davis Parker, Corrado Angelini

*Corresponding author for this work

Research output: Contribution to journalArticlepeer-review

155 Scopus citations

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 languageEnglish (US)
Pages (from-to)1041-1045
Number of pages5
JournalNeurology
Volume35
Issue number7
DOIs
StatePublished - Jul 1985

ASJC Scopus subject areas

  • Clinical Neurology

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