Coenzyme a Biochemistry: From Neurodevelopment to Neurodegeneration

Coenzyme A (CoA) is an essential cofactor in all living organisms. It is involved in a large number of biochemical processes functioning either as an activator of molecules with carbonyl groups or as a carrier of acyl moieties. Together with its thioester derivatives, it plays a central role in cell...

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Published inBrain sciences Vol. 11; no. 8; p. 1031
Main Authors Mignani, Luca, Gnutti, Barbara, Zizioli, Daniela, Finazzi, Dario
Format Journal Article
LanguageEnglish
Published Basel MDPI AG 02.08.2021
MDPI
Subjects
Bacteria
Biochemistry
Biosynthesis
Carbonyl compounds
Cellular stress response
coasy
Coenzyme A
COPAN
Enzymes
Gene expression
Homeostasis
Kinases
Localization
Mammals
Metabolism
Metabolites
Mutation
Neurodegeneration
Neurodegenerative diseases
Neurodevelopment
Neurodevelopmental disorders
Oxidative stress
pank2
Phosphorylation
PKAN
Post-translation
Proteins
Review
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Summary:Coenzyme A (CoA) is an essential cofactor in all living organisms. It is involved in a large number of biochemical processes functioning either as an activator of molecules with carbonyl groups or as a carrier of acyl moieties. Together with its thioester derivatives, it plays a central role in cell metabolism, post-translational modification, and gene expression. Furthermore, recent studies revealed a role for CoA in the redox regulation by the S-thiolation of cysteine residues in cellular proteins. The intracellular concentration and distribution in different cellular compartments of CoA and its derivatives are controlled by several extracellular stimuli such as nutrients, hormones, metabolites, and cellular stresses. Perturbations of the biosynthesis and homeostasis of CoA and/or acyl-CoA are connected with several pathological conditions, including cancer, myopathies, and cardiomyopathies. In the most recent years, defects in genes involved in CoA production and distribution have been found in patients affected by rare forms of neurodegenerative and neurodevelopmental disorders. In this review, we will summarize the most relevant aspects of CoA cellular metabolism, their role in the pathogenesis of selected neurodevelopmental and neurodegenerative disorders, and recent advancements in the search for therapeutic approaches for such diseases.
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ISSN:2076-3425
2076-3425
DOI:10.3390/brainsci11081031