Journal article
Dicarboxylic Aciduria: Deficient [1- 14 C]Octanoate Oxidation and Medium-Chain Acyl-CoA Dehydrogenase in Fibroblasts
Science (American Association for the Advancement of Science), Vol.221(4605), pp.73-75
07/1983
DOI: 10.1126/science.6857268
PMID: 6857268
Abstract
Dicarboxylic aciduria, an inborn error of metabolism in man, is thought to be caused by defective β-oxidation of six-carbon to ten-carbon fatty acids. Oxidation of [1-14C]octanoate was impaired in intact fibroblasts from three unrelated patients with dicarboxylic aciduria (19 percent of control), as was the activity of medium-chain (octanoyl-)acyl-CoA dehydrogenase in the supernatants of sonicated fibroblast mitochondria (5 percent of control). These data confirm that dicarboxylic aciduria is caused by an enzyme defect in the β-oxidation cycle.
Details
- Title: Subtitle
- Dicarboxylic Aciduria: Deficient [1- 14 C]Octanoate Oxidation and Medium-Chain Acyl-CoA Dehydrogenase in Fibroblasts
- Creators
- William J. Rhead - University of IowaBrad A. Amendt - University of IowaKathie S. Fritchman - University of IowaSara J. Felts - University of Iowa
- Resource Type
- Journal article
- Publication Details
- Science (American Association for the Advancement of Science), Vol.221(4605), pp.73-75
- DOI
- 10.1126/science.6857268
- PMID
- 6857268
- NLM abbreviation
- Science
- ISSN
- 0036-8075
- eISSN
- 1095-9203
- Language
- English
- Date published
- 07/1983
- Academic Unit
- Orthodontics; Anatomy and Cell Biology; Craniofacial Anomalies Research Center; Dental Research
- Record Identifier
- 9984284347902771
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