CARNITINE REDUCES FASTING KETOGENESIS IN PATIENTS WITH DISORDERS OF PROPIONATE METABOLISM

Jon A. Wolff, Le Phuc Thuy, Richard Haas, James Edwin Carroll, Christina Prodanos, William L. Nyhan

Research output: Contribution to journalArticlepeer-review

23 Scopus citations

Abstract

Patients with disorders of propionate metabolism have low plasma levels of free carnitine and excrete higher than normal quantities of esterified carnitine. The response to a 19 h fast was assessed as a physiological index of carnitine deficiency. In patients with propionic acidaemia and methylmalonic acidaemia a substantial ketogenesis developed in response to fasting. Supplementation with L-carnitine significantly reduced this ketogenic response.

Original languageEnglish (US)
Pages (from-to)289-291
Number of pages3
JournalThe Lancet
Volume327
Issue number8476
DOIs
StatePublished - Feb 8 1986

ASJC Scopus subject areas

  • Medicine(all)

Fingerprint Dive into the research topics of 'CARNITINE REDUCES FASTING KETOGENESIS IN PATIENTS WITH DISORDERS OF PROPIONATE METABOLISM'. Together they form a unique fingerprint.

Cite this