1. Hereditary diseases of coenzyme A thioester metabolism
- Author
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Chen Zhao, Shu Pei Wang, Grant A. Mitchell, Hao Yang, and Youlin Wang
- Subjects
chemistry.chemical_classification ,0303 health sciences ,Newborn screening ,Coenzyme A ,Infant, Newborn ,Metabolism ,Thioester ,Biochemistry ,Pathophysiology ,03 medical and health sciences ,chemistry.chemical_compound ,Neonatal Screening ,0302 clinical medicine ,chemistry ,Toxicity ,Hereditary Diseases ,Animals ,Humans ,Redistribution (chemistry) ,Acyl Coenzyme A ,030217 neurology & neurosurgery ,030304 developmental biology - 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).
- Published
- 2019
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