Markus Andreas Keller

Beyond the surface: plasmalogens are dispensable for retinal integrity and fertility in the mouse

This study compares Gnpat and Peds1 knockout mice to determine the role of plasmalogens in retinal integrity and fertility, finding that plasmanyl lipids can compensate for plasmalogen loss.

Disturbed mitochondrial maturation in cardiolipin remodeling-deficient cardiomyocytes

This study shows that TAFAZZIN deficiency disrupts mitochondrial maturation in cardiomyocytes, leading to structural and functional impairments associated with Barth syndrome cardiomyopathy.

Biochemical signatures from dried blood spots in untargeted metabolics are influenced by matrix effects

The paper investigates matrix effects on metabolite recovery from dried blood spots (DBS) and shows that despite these effects, DBS can be used for diagnostics with proper validation.

Trapped ion mobility spectrometry-guided molecular discrimination between plasmalogens and other ether lipids in lipidomics experiments

This paper demonstrates that ion mobility spectrometry can distinguish between plasmalogens and other ether lipids by analyzing their gas-phase geometries, improving lipid annotation accuracy.

Stem cell models of TAFAZZIN deficiency reveal novel tissue-specific pathologies in Barth Syndrome

This study uses stem cell models to investigate tissue-specific pathologies in Barth Syndrome caused by TAFAZZIN deficiency, identifying mitophagy defects and potential therapeutic targets.

Mitochondrial cardiolipin metabolism controlled by tafazzin enables ferroptosis

The paper shows that tafazzin deficiency in Barth Syndrome protects against ferroptosis by altering mitochondrial cardiolipin metabolism, affecting VDAC function and reducing ROS availability for ferroptosis.