Short-chain acyl-coenzyme A dehydrogenase deficiency in mice.
Publication
, Journal Article
Wood, PA; Amendt, BA; Rhead, WJ; Millington, DS; Inoue, F; Armstrong, D
Published in: Pediatr Res
January 1989
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.
Duke Scholars
Altmetric Attention Stats
Dimensions Citation Stats
Published In
Pediatr Res
DOI
ISSN
0031-3998
Publication Date
January 1989
Volume
25
Issue
1
Start / End Page
38 / 43
Location
United States
Related Subject Headings
- Succinates
- Pediatrics
- Oxidation-Reduction
- Mice, Inbred BALB C
- Mice
- Malonates
- Glycine
- Gas Chromatography-Mass Spectrometry
- Fatty Acids, Volatile
- Fatty Acids
Citation
APA
Chicago
ICMJE
MLA
NLM
Wood, P. A., Amendt, B. A., Rhead, W. J., Millington, D. S., Inoue, F., & Armstrong, D. (1989). Short-chain acyl-coenzyme A dehydrogenase deficiency in mice. Pediatr Res, 25(1), 38–43. https://doi.org/10.1203/00006450-198901000-00010
Wood, P. A., B. A. Amendt, W. J. Rhead, D. S. Millington, F. Inoue, and D. Armstrong. “Short-chain acyl-coenzyme A dehydrogenase deficiency in mice.” Pediatr Res 25, no. 1 (January 1989): 38–43. https://doi.org/10.1203/00006450-198901000-00010.
Wood PA, Amendt BA, Rhead WJ, Millington DS, Inoue F, Armstrong D. Short-chain acyl-coenzyme A dehydrogenase deficiency in mice. Pediatr Res. 1989 Jan;25(1):38–43.
Wood, P. A., et al. “Short-chain acyl-coenzyme A dehydrogenase deficiency in mice.” Pediatr Res, vol. 25, no. 1, Jan. 1989, pp. 38–43. Pubmed, doi:10.1203/00006450-198901000-00010.
Wood PA, Amendt BA, Rhead WJ, Millington DS, Inoue F, Armstrong D. Short-chain acyl-coenzyme A dehydrogenase deficiency in mice. Pediatr Res. 1989 Jan;25(1):38–43.
Published In
Pediatr Res
DOI
ISSN
0031-3998
Publication Date
January 1989
Volume
25
Issue
1
Start / End Page
38 / 43
Location
United States
Related Subject Headings
- Succinates
- Pediatrics
- Oxidation-Reduction
- Mice, Inbred BALB C
- Mice
- Malonates
- Glycine
- Gas Chromatography-Mass Spectrometry
- Fatty Acids, Volatile
- Fatty Acids