Journal article
Short-Chain Acyl-Coenzyme A Dehydrogenase Deficiency in Mice
Pediatric research, Vol.25(1), pp.38-43
01/1989
DOI: 10.1203/00006450-198901000-00010
PMID: 2919115
Abstract
A murine model for short-chain acyl-coen-zyme 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 β-oxidation defects. This mouse model presents important opportunities to investigate the biology of mammalian fatty acid metabolism and the related human diseases. © 1989 International Pediatric Research Foundation, Inc.
Details
- Title: Subtitle
- Short-Chain Acyl-Coenzyme A Dehydrogenase Deficiency in Mice
- Creators
- Philip A Wood - Baylor College of MedicineBrad A Amendt - University of IowaWilliam J Rhead - University of IowaDavid S Millington - Duke UniversityFumio Inoue - Duke UniversityDawna Armstrong - Baylor College of Medicine
- Resource Type
- Journal article
- Publication Details
- Pediatric research, Vol.25(1), pp.38-43
- DOI
- 10.1203/00006450-198901000-00010
- PMID
- 2919115
- NLM abbreviation
- Pediatr Res
- ISSN
- 0031-3998
- eISSN
- 1530-0447
- Language
- English
- Date published
- 01/1989
- Academic Unit
- Orthodontics; Anatomy and Cell Biology; Craniofacial Anomalies Research Center; Dental Research
- Record Identifier
- 9984284449302771
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