ACADL


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ACADL

A gene on chromosome 2q34-q35 that encodes an acyl-CoA dehydrogenase which is a mitochondrial flavoenzyme involved in fatty acid and branched chain amino-acid metabolism. It is one of four enzymes that catalyse the first step of mitochondrial beta-oxidation of straight-chain fatty acid.

Molecular pathology
Defects of ACADL cause long-chain acyl-CoA dehydrogenase (LCAD) deficiency, leading to nonketotic hypoglycaemia.
References in periodicals archive ?
Activation of PPARG promotes terminal differentiation through the induction of a range of genes important for TG formation through fatty acid oxidation among downstream pathways involving CPT2, ACADL, and ACOX2.
These included triglyceride hydrolysis (PNPLA2), FA esterification (ACSL1), mitochondrial import (CPT1A, CPT1B, and SLC25A20) and [beta]-oxidation (ACADL, ACADVL, HADHA, and HADHB).
This conversion is catalyzed by numerous enzymes, including Acadl, Acadvl, Hadha, Hadhb, Decr1, and Acaa2.