Defects in mitochondrial beta-oxidation of fatty acids

Authors
Wood, PA
Citation
Pa. Wood, Defects in mitochondrial beta-oxidation of fatty acids, CURR OP LIP, 10(2), 1999, pp. 107-112
Citations number
41
Categorie Soggetti
Cardiovascular & Hematology Research
Journal title
CURRENT OPINION IN LIPIDOLOGY
ISSN journal
09579672 → ACNP
Volume
10
Issue
2
Year of publication
1999
Pages
107 - 112
Database
ISI
SICI code
0957-9672(199904)10:2<107:DIMBOF>2.0.ZU;2-9
Abstract
Mitochondrial beta-oxidation of fatty acids generates energy by direct elec tron transfer at the dehydrogenase steps along with the ultimate product of acetyl-coenzyme A that can be further oxidized for ATP synthesis, or conve rsion to ketone bodies. This review describes the human inborn errors of th is pathway and recent results concerning the development and use of mouse m odels of these inherited enzyme deficiencies. Curr Opin Lipidol 10:107-112. (C) 1999 Lippincott Williams & Wilkins.