Hereditary diseases of coenzyme A thioester metabolism. (9th January 2019)

• Record Type: Journal Article
• Authors: Yang, Hao; Zhao, Chen; Wang, Youlin; Wang, Shu Pei; Mitchell, Grant A.
• Is Part Of: Biochemical Society transactions. Volume 47:Number 1(2019)
• Page Start: 149
• Page End: 155
• Publication Date: 2019-01-09
• Subjects: CoA thioester -- inborn error -- pathophysiology; Biochemistry -- Congresses; 572
• DOI: 10.1042/BST20180423
• Languages: English
• ISSNs: 0300-5127

Abstract

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).