X-linked Adrenoleukodystrophy
X-linked Adrenoleukodystrophy (X-ALD), the most common peroxisomal disorder, is an inherited neurodegenerative disorder that is associated with inflammation and irreversible destruction of myelin in the brain. We are using cell culture and mouse models that lack functional ALD protein to study the basic pathomechanisms of X-ALD and to test novel molecular strategies for therapeutic intervention (pharmacological gene therapy).
Functions of Peroxisomes in the Central Nervous System
We are interested in the proteom of peroxisomes in different tissues, in order to elucidate novel peroxisomal pathways and functions important for the CNS. In addition, we have generated a transgenic mouse model with green fluorescent peroxisomes which, together with other mouse models are used to explain specific questions concerning the peroxisomes and the nervous system.
Metachromatic Leukodystrophy
Metachromatic leukodystrophy is an inherited sulfatide storage disease, classically due to deficiency of the lysosomal enzyme arylsulfatase A. We have been interested in the genotype-phenotype relation in this leukodystrophy -more-
