L-carnitine enhances excretion of propionyl coenzyme A as propionylcarnitine in propionic acidemia.

Published

Journal Article

Treatment with L-carnitine greatly enhanced the formation and excretion of short-chain acylcarnitines in three patients with propionic acidemia and in three normal controls. The use of fast atom bombardment mass spectrometry and linked scanning at constant magnetic (B) to electric (E) field ratio identified the acylcarnitine as propionylcarnitine in patients with propionic acidemia. The normal children excreted mostly acetylcarnitine. Propionic acidemia and other organic acidurias are characterized by the intramitochondrial accumulation of short-chain acyl-Coenzyme A (CoA) compounds. The substrate specificity of the carnitine acetyltransferase enzyme and its steady state nature appears to facilitate elimination of propionyl groups while restoring the acyl-CoA:free CoA ratio in the mitochondrion. We suggest that L-carnitine may be a useful therapeutic approach for elimination of toxic acyl CoA compounds in several of these disorders.

Full Text

Duke Authors

Cited Authors

  • Roe, CR; Millington, DS; Maltby, DA; Bohan, TP; Hoppel, CL

Published Date

  • June 1, 1984

Published In

Volume / Issue

  • 73 / 6

Start / End Page

  • 1785 - 1788

PubMed ID

  • 6725560

Pubmed Central ID

  • 6725560

International Standard Serial Number (ISSN)

  • 0021-9738

Digital Object Identifier (DOI)

  • 10.1172/JCI111387

Language

  • eng

Conference Location

  • United States