Short-chain acyl-coenzyme A dehydrogenase deficiency in mice.

Journal Article (Journal Article)

A murine model for short-chain acyl-coenzyme A dehydrogenase (SCAD) deficiency has been identified and characterized in BALB/cByJ mice. These mice have undetectable SCAD activity, severe organic aciduria; excreting ethylmalonic and methylsuccinic acids and N-butyrylglycine, and develop a fatty liver upon fasting or dietary fat challenge. The mutant mice develop hypoglycemia after an 18-h fast, and have elevated urinary and muscle butyrylcarnitine concentrations. Most of these findings parallel those of human disorders associated with SCAD deficiency and other beta-oxidation defects. This mouse model presents important opportunities to investigate the biology of mammalian fatty acid metabolism and the related human diseases.

Full Text

Duke Authors

Cited Authors

  • Wood, PA; Amendt, BA; Rhead, WJ; Millington, DS; Inoue, F; Armstrong, D

Published Date

  • January 1989

Published In

Volume / Issue

  • 25 / 1

Start / End Page

  • 38 - 43

PubMed ID

  • 2919115

International Standard Serial Number (ISSN)

  • 0031-3998

Digital Object Identifier (DOI)

  • 10.1203/00006450-198901000-00010


  • eng

Conference Location

  • United States