Disturbed mitochondrial maturation in cardiolipin remodeling-deficient cardiomyocytes

iScience 2026 Open access

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Authors: Nanami Senoo, Macie S. Sheridan, Yvonne Wohlfarter, Mackenzie T. Primrose, Emmanouil Tampakakis, Markus A. Keller, Steven M. Claypool
DOI: 10.1016/j.isci.2026.115111
Cited by: 4
References: 82

Abstract

maturation treatments that simulate heart developmental stimuli. We found that cardiomyocyte maturation involves progressive cristae dynamics associated with protein and lipid alterations in the inner mitochondrial membrane. TAFAZZIN-deficient cardiomyocytes fail to adapt to the developmental stimuli, resulting in damaged cristae, compromised mitochondrial respiration, and cardiomyocyte dysfunction. These results demonstrate that TAFAZZIN deficiency perturbs functional and structural development of mitochondria, which may contribute to mitochondrial dysfunction and associated childhood progression to cardiomyopathy in Barth syndrome.

Fields of Study & Subjects

Mitochondrial Function and Pathology Cardiomyopathy and Myosin Studies Congenital heart defects research

Publication details

Journal / source
iScience
Publisher
Elsevier BV
Volume
29
Issue
3
Pages
115111–115111
Publication date
20 February 2026
Language
EN