Research - Keller Group

Research Focus Membrane lipid metabolism of organelles and cells Regulation and compartmentalization of cellular lipid metabolism Pathobiochemisty of inborn errors in mitochondrial phospholipid homeostasis Special focus on disease affecting cardiolipins including Barth Syndrome, MEGDEL Syndrome and Sengers Syndrome Functionally linking genetic alterations with metabolic reconfigurations in patient derived fibroblasts and CRISPR/Cas9  knock-out cell lines Novel analytical, data analysis, and bioinformatics approaches Development of liquid chromatography – tandem mass spectrometric (LC-MS/MS) metabolomics and lipidomic methodology. Novel analysis strategies for high content mass spectrometric data (e.g. Lipid class enrichment analysis, mathematical lipid subspecies modeling from MS/MS data) Membrane enzyme structure and ligand interactions In vivo quantification of fatty aldehyde metabolism in Sjögren Larsson Syndrome patient cells X-ray structure analysis of fatty aldehyde dehydrogenase and Sjögren Larsson Syndrome causing mutations in this enzyme Identification and characterization of pharmacological chaperone ligands as potential treatment option for Sjögren Larsson Syndrome patients