| long-chain a. (LCAD) deficiency |
a defect in mitochondrial beta oxidation due to deficiency of the acyl-CoA dehydrogenase acting on long chain length fatty acids. It is clinically similar to MCAD deficiency, but urinary excretion is of long-chain dicarboxylic acids and skeletal muscle weakness and cardiac enlargement may also be present.
출처: www.mercksource.com/pp/us/cns/cns_health_library.j...
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| long-chain-fatty-acid–CoA ligase |
[EC 6.2.1.3] an enzyme of the ligase class that catalyzes the formation of acyl coenzyme A from long chain fatty acids (12 or more carbons) and coenzyme A, using energy derived from ATP hydrolysis. The enzyme occurs in the mitochondrial outer membrane, endoplasmic reticulum, and peroxisome membrane, and it acts on saturated and unsaturated fatty acids as well as some hydroxy acids. Called also acyl CoA synthetase.
출처: www.mercksource.com/pp/us/cns/cns_health_library.j...
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