| First Author | Wood PA | Year | 1989 |
| Journal | Pediatr Res | Volume | 25 |
| Issue | 1 | Pages | 38-43 |
| PubMed ID | 2919115 | Mgi Jnum | J:14707 |
| Mgi Id | MGI:62870 | Doi | 10.1203/00006450-198901000-00010 |
| Citation | Wood PA, et al. (1989) Short-chain acyl-coenzyme A dehydrogenase deficiency in mice. Pediatr Res 25(1):38-43 |
| abstractText | 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. |
