1. Hereditary diseases of coenzyme A thioester metabolism.
- Author
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Hao Yang, Chen Zhao, Youlin Wang, Shu Pei Wang, and Mitchell, Grant A.
- Subjects
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COENZYME A , *GENETIC disorders , *BIOCHEMICAL genetics , *DEHYDROGENASE genetics , *MITOCHONDRIA - 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). [ABSTRACT FROM AUTHOR]
- Published
- 2019
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