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Research - Keller Group
Research FocusMembrane lipid metabolism of organelles and cellsRegulation and compartmentalization of cellular lipid metabolismPathobiochemisty of inborn errors in mitochondrial phospholipid homeostasis Special focus on disease affecting cardiolipins including Barth Syndrome, MEGDEL Syndrome and Sengers SyndromeFunctionally linking genetic alterations with metabolic reconfigurations in patient derived fibroblasts and CRISPR/Cas9 knock-out cell linesNovel analytical, data analysis, and bioinformatics approachesDevelopment 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 interactionsIn vivo quantification of fatty aldehyde metabolism in Sjögren Larsson Syndrome patient cellsX-ray structure analysis of fatty aldehyde dehydrogenase and Sjögren Larsson Syndrome causing mutations in this enzymeIdentification and characterization of pharmacological chaperone ligands as potential treatment option for Sjögren Larsson Syndrome patients