Phosphatidylglycerol Supplementation Alters Mitochondrial Morphology and Cardiolipin Composition.

ABSTRACT: The pathogenic variant of the TAZ gene is directly associated with Barth syndrome. Because tafazzin in the mitochondria is responsible for cardiolipin (CL) remodeling, all molecules related to the metabolism of CL can affect or be affected by TAZ mutation. In this study, we intend to recover the distortion of the mitochondrial lipid composition, especially CL, for Barth syndrome treatment. The genetically edited TAZ knockout HAP1 cells were demonstrated to be a suitable cellular model, where CL desaturation occurred and monolyso-CL (MLCL) was accumulated. From the species analysis by mass spectrometry, phosphatidylethanolamine showed changed species content after TAZ knockout. TAZ knockout also caused genetic down-regulation of PGS gene and up-regu

SUBMITTER: Chu I 

PROVIDER: S-EPMC9028734 | biostudies-literature | 2022 Mar

REPOSITORIES: biostudies-literature