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Comment
. 2025 Nov;98(5):1621-1622.
doi: 10.1038/s41390-025-04278-5. Epub 2025 Jul 9.

Patient-derived induced pluripotent stem cell models reveal mechanistic links between aberrant mitochondrial dynamics and cardiomyopathy

Chrishan J Ramachandra  1   2
Affiliations
Comment

Patient-derived induced pluripotent stem cell models reveal mechanistic links between aberrant mitochondrial dynamics and cardiomyopathy

Chrishan J Ramachandra. Pediatr Res. 2025 Nov.

Abstract

DNM1L mutations impair mitochondrial fission, leading to cardiomyocyte energy deficits and contractile dysfunction, and reveal a cardiac role for DNM1L beyond neurological disease. iPSC-cardiomyocytes derived from patients with DNM1L mutations demonstrate mitochondrial defects and cardiomyopathy phenotypes, offering a robust model to dissect disease mechanisms and identify personalised therapies. Disrupted mitochondrial dynamics directly lead to calcium mishandling and contractile dysfunction, positioning fission/fusion pathways as promising therapeutic targets in cardiomyopathy treatment.

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Conflict of interest statement

Competing interests: The author declares no competing interests.

Comment on

References

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