Article

Barth syndrome cellular models have dysregulated respiratory chain complex I and mitochondrial quality control due to abnormal cardiolipin

2021-01-08

Abstract excerpt

Barth syndrome (BTHS) is an X-linked genetic condition caused by defects in TAZ , which encodes a transacylase involved in the remodeling of the inner mitochondrial membrane phospholipid, cardiolipin (CL). As such, CL has been implicated in numerous mitochondrial functions, and the role of defective CL in the clinical pathology of BTHS is under intense investigation. We used untargeted proteomics, shotgun lipidom...

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Literature Corpus work: cdc87aef-87db-59ae-a16a-2a5ff19f65cb
DOI: 10.1101/2021.01.06.425502

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