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

*Corresponding author for this work

Research output: Contribution to journalArticleAcademicpeer-review

3 Citations (Scopus)
44 Downloads (Pure)

Abstract

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.

Original languageEnglish
Pages (from-to)885-894
Number of pages10
JournalJournal of Inherited Metabolic Disease
Volume47
Issue number5
Early online date9-Apr-2024
DOIs
Publication statusPublished - 2024

Keywords

  • 4′-phosphopantetheine
  • cardiac dysfunction
  • coenzyme A (CoA)
  • heart failure (HF)
  • pantethine
  • PKAN
  • PPCDC DD
  • PPCS DD
  • TANGO2 DD
  • Type II 3-methylglutaconic aciduria
  • vitamin B5 (or pantothenic acid)

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