| Literature DB >> 2868163 |
J A Wolff, J E Carroll, C Prodanos, R Haas, W L Nyhan.
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.Entities:
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Year: 1986 PMID： 2868163 DOI： 10.1016/s0140-6736(86)90826-3
Source DB: PubMed Journal: Lancet ISSN： 0140-6736 Impact factor: 79.321