Journal article
Short-Chain Acyl-Coenzyme A Dehydrogenase Activity, Antigen, and Biosynthesis Are Absent in the BALB/cByJ Mouse
Pediatric research, Vol.31(6), pp.552-556
06/1992
DOI: 10.1203/00006450-199206000-00003
PMID: 1635815
Abstract
BALB/cByJ (J) mice have short-chain acyl-CoA dehydrogenase (SCAD) deficiency and an organic aciduria similar to that of human SCAD deficiency. [9,10(n)-3H]- and [15,16(n)-3Hlpalmitate oxidations in J mouse fibroblasts were 96 and 35% of control, respectively, consistent with an isolated SCAD defect. Acyl-CoA dehydrogenase activities were assayed in muscle and fibroblast mitochondria from BALB/cBy controls (Y) and SCAD-deficient J mice. Medium-chain acyl-CoA dehydrogenase (MCAD) activities were comparable in both J and Y mice from all tissues. In the presence of MCAD antiserum, SCAD activities in J mice were undetectable in both tissues. Apparent Km and Vraax values in liver mitochondria suggested a somewhat increased affinity of MCAD for butyryl-CoA in J mice, as compared with MCAD from other species. Immunoblot studies using mitochondria revealed identical apparent SCAD molecular weight in liver, muscle, and fibroblasts from Y mice and no detectable SCAD antigen in J mice; MCAD antigen was detected in comparable amounts from both Y and J mice. Radiolabel-ing and immunoprecipitation studies in J mouse fibroblasts revealed no SCAD synthesis, but normal MCAD synthesis. These data argue against the existence of tissue-specific SCAD isoforms in the mouse and confirm that this mouse strain is a model for the human organic aciduria resulting from this β-oxidation defect. © 1992 International Pediatric Research Foundation, Inc.
Details
- Title: Subtitle
- Short-Chain Acyl-Coenzyme A Dehydrogenase Activity, Antigen, and Biosynthesis Are Absent in the BALB/cByJ Mouse
- Creators
- Brad A Amendt - University of IowaEric Freneaux - University of IowaCameron Reece - University of IowaPhilip A Wood - University of Alabama at BirminghamWilliam J Rhead - University of Iowa
- Resource Type
- Journal article
- Publication Details
- Pediatric research, Vol.31(6), pp.552-556
- DOI
- 10.1203/00006450-199206000-00003
- PMID
- 1635815
- NLM abbreviation
- Pediatr Res
- ISSN
- 0031-3998
- eISSN
- 1530-0447
- Language
- English
- Date published
- 06/1992
- Academic Unit
- Orthodontics; Anatomy and Cell Biology; Craniofacial Anomalies Research Center; Dental Research
- Record Identifier
- 9984284323402771
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