The complexity of cardiolipin in health and disease

Steven M. Claypool, Carla M. Koehler

Research output: Contribution to journalReview articlepeer-review


Cardiolipin, the signature phospholipid of mitochondria, is a lipid dimer that is important for a diverse range of mitochondrial activities beyond the process of ATP production. Thus not surprisingly, derangements in cardiolipin metabolism are now appreciated to contribute to an assortment of pathological conditions. A comprehensive inventory of enzymes involved in cardiolipin biosynthesis and remodeling was just recently obtained. Post-biosynthesis, the acyl chain composition of cardiolipin is modified by up to three distinct remodeling enzymes that produce either a homogeneous tissue-specific mature form of cardiolipin or alternatively 'bad' cardiolipin that has been linked to mitochondrial dysfunction. In this review, we initially focus on the newly identified players in cardiolipin metabolism and then shift our attention to how changes in cardiolipin metabolism contribute to human disease.

Original languageEnglish (US)
Pages (from-to)32-41
Number of pages10
JournalTrends in biochemical sciences
Issue number1
StatePublished - Jan 2012

ASJC Scopus subject areas

  • Biochemistry
  • Molecular Biology


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