Disturbed mitochondrial maturation in cardiolipin remodeling-deficient cardiomyocytes

Abstract excerpt

<h4>Summary</h4> Barth syndrome, a rare X-linked genetic disorder, features early-onset cardiomyopathy. The causal gene, TAFAZZIN , encodes a transacylase that mediates acyl chain remodeling of cardiolipin, a critical phospholipid in the inner mitochondrial membrane. While Barth syndrome exhibits hallmark cardiolipin abnormalities, the precise mechanisms linking TAFAZZIN deficiency and disturbed cardiolipin meta...

Topics

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• Cardiolipins
• Cardiomyopathies
• Disease Models, Animal
• Heart Failure
• Mice
• Mice, Knockout
• Mitochondria
• Transcription Factors

Identifiers and source

Literature Corpus work

29fe1e1a-8ef9-57ac-9d2c-63948f25c791

DOI

10.1101/2025.08.07.668981