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Wedman, J.J.* ; Sibon, O.C.M.* ; Mastantuono, E.* ; Iuso, A.

Impaired coenzyme A homeostasis in cardiac dysfunction and benefits of boosting coenzyme A production with vitamin B5 and its derivatives in the management of heart failure.

J. Inherit. Metab. Dis., DOI: 10.1002/jimd.12737 (2024)
DOI PMC
Creative Commons Lizenzvertrag
Coenzyme A (CoA) is an essential cofactor required for over a hundred metabolic reactions in the human body. This cofactor is synthesized de novo in our cells from vitamin B5, also known as pantothenic acid, a water-soluble vitamin abundantly present in vegetables and animal-based foods. Neurodegenerative disorders, cancer, and infectious diseases have been linked to defects in de novo CoA biosynthesis or reduced levels of this coenzyme. There is now accumulating evidence that CoA limitation is a critical pathomechanism in cardiac dysfunction too. In the current review, we will summarize our current knowledge on CoA and heart failure, with emphasis on two primary cardiomyopathies, phosphopantothenoylcysteine synthetase and phosphopantothenoylcysteine decarboxylase deficiency disorders biochemically characterized by a decreased level of CoA in patients' samples. Hence, we will discuss the potential benefits of CoA restoration in these diseases and, more generally, in heart failure, by vitamin B5 and its derivatives pantethine and 4'-phosphopantetheine.
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Publication type Article: Journal article
Document type Scientific Article
Corresponding Author
Keywords 4′‐phosphopantetheine ; Pkan ; Ppcdc Dd ; Ppcs Dd ; Tango2 Dd ; Type Ii 3‐methylglutaconic Aciduria ; Cardiac Dysfunction ; Coenzyme A (coa) ; Heart Failure (hf) ; Pantethine ; Vitamin B5 (or Pantothenic Acid); Pantothenic-acid; Fatty-acid; Pantethine Rescues; Escherichia-coli; Metabolic Crises; Rat; Deficiency; Coa; 4-phosphopantetheine; Cardiomyopathy
ISSN (print) / ISBN 0141-8955
e-ISSN 1573-2665
Journal Journal of Inherited Metabolic Disease
Publisher Springer
Publishing Place 111 River St, Hoboken 07030-5774, Nj Usa
Non-patent literature Publications
Reviewing status Peer reviewed
Institute(s) Institute of Neurogenomics (ING)
Grants NBIA Disorders Association
NBIA Suisse
Hoffnungsbaum e.V.
NBIA Poland