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Updated: Jan 31, 2026

Quantification of Coenzyme A in Cells and Tissues
Published on: September 27, 2019
Hereditary diseases of coenzyme A thioester metabolism
Hao Yang1, Chen Zhao1,2, Youlin Wang1
1Division of Medical Genetics, Department of Pediatrics, CHU Sainte-Justine and Université de Montréal, Montréal, QC, Canada.
Abstract:
Coenzyme A (CoA) thioesters (acyl-CoAs) are essential intermediates of metabolism. Inborn errors of acyl-CoA metabolism include a large fraction of the classical organic acidemias. These conditions can involve liver, muscle, heart and brain, and can be fatal. These conditions are increasingly detected by newborn screening. There is a renewed interest in CoA metabolism and in developing effective new treatments. Here, we review theories of the pathophysiology in relation to mitochondrial CoA sequestration, toxicity and redistribution (CASTOR).
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