Institute of Experimental Medicine CAS

Blocking phosphatidylglycerol degradation in yeast defective in cardiolipin remodeling results in a new model of the Barth syndrome cellular phenotype

 

Barth syndrome is an inherited mitochondrial disorder characterized by misregulation of lipid synthesis. The molecular basis of the pathology is not well understood. We created a new yeast strain to better approximate human Barth syndrome cells. We show that our strain has more pronounced mitochondrial respiratory defects and a higher number of abnormal mitochondria compared to the yeast strain used previously as the Barth syndrome model. We further show that the mood-stabilizing anticonvulsant valproic acid has a positive effect on both lipid composition and mitochondrial function in the yeast Barth syndrome models. Our newly created yeast model of human Barth syndrome cells better mimics the cellular phenotype of Barth syndrome patients than the strain used previously. This favors the new model for use in future studies.

 

The article was written in collaboration with the Institute of Animal Biochemistry and Genetics SAS and is available as Open Access.