Division of Medical Cell Biology
Hepatocyte as an example for a eukaryotic cell |
…working on
Peroxisomes in Biology and Medicine
Eukaryotic cells contain distinct intracellular compartments - called
organelles - involved in different metabolic pathways.
Cell organelles are working together to keep the cells healthy (regulating
homeostasis) and protect them against extra- and intra-cellular toxic
events leading to cell death.
Peroxisomes are single membrane-bound organelles, ubiquitously expressed in all tissues, protecting cells against toxic oxygen species and bioactive as well as toxic lipid derivatives. In addition, peroxisomes are intimately involved in the synthesis of special membrane lipids to guarantee the normal function of the plasma membrane.
The ultimate importance of peroxisomes for human health is stressed by the devastating diseases resulting from defects in the biogenesis of this organelle.
The complete loss of peroxisomal metabolic pathways in peroxisomal biogenesis disorders is caused by mutations/deletions in peroxin genes (PEX genes), whose protein products are involved in the regular biogenesis of peroxisomes. Children with Zellweger syndrome - the most severe form of the peroxisomal biogenesis disorders – suffer from generalized hypotonia and neonatal seizures. Zellweger patients have migration defects of neurons in the central nervous system and develop degenerative organ defects (liver fibrosis/ cirrhosis, degeneration of the adrenal cortex…) and die often during the first year of life.
We are currently working on appropriate transgenic animals (PEX-knockouts) and different cell culture systems as experimental models for studying the molecular pathogenesis of organ defects in these diseases and to possibly find a strategy to cure the patients.
Have a look at our
projects and the people involved…
PhD (Doctoral)-or Master (Diploma)-Thesis
Impressionen: Laboreinweihung