Hereditary diseases of coenzyme A thioester metabolism-Reference-Cited by-同舟云学术

Hereditary diseases of coenzyme A thioester metabolism

Published:2019-01-09 Issue:1 Volume:47 Page:149-155
ISSN:0300-5127
Container-title:Biochemical Society Transactions
language:en
Short-container-title:

Author:

Yang Hao¹,Zhao Chen¹²,Wang Youlin¹,Wang Shu Pei¹,Mitchell Grant A.¹^ORCID

Affiliation:

1. Division of Medical Genetics, Department of Pediatrics, CHU Sainte-Justine and Université de Montréal, Montréal, QC, Canada

2. College of Animal Science and Technology, Northwest A&F University, Yangling, Shaanxi, China

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).

Publisher

Portland Press Ltd.

Subject

Biochemistry

Link

https://portlandpress.com/biochemsoctrans/article-pdf/47/1/149/848708/bst-2018-0423c.pdf

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