@article{78b2aa765e5e4dcf8b14dec2fa3b81ff,
title = "CARNITINE REDUCES FASTING KETOGENESIS IN PATIENTS WITH DISORDERS OF PROPIONATE METABOLISM",
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.",
author = "Wolff, {Jon A.} and Thuy, {Le Phuc} and Richard Haas and Carroll, {James Edwin} and Christina Prodanos and Nyhan, {William L.}",
note = "Funding Information: New Jersey. This work was supported by National Institutes of Health grants HD040608 from the National Institute of Child Health and Human Development, AM07318 from the National Institute of Arthritis, Diabetes, Digestive and Kidney Diseases, and RR00827 from the General Clinical Research Center Program of the Division of Research Resources.",
year = "1986",
month = feb,
day = "8",
doi = "10.1016/S0140-6736(86)90826-3",
language = "English (US)",
volume = "327",
pages = "289--291",
journal = "The Lancet",
issn = "0140-6736",
publisher = "Elsevier Limited",
number = "8476",
}