Department(s): Department of Pathobiology of the Nervous System (Center for Brain Research)
Position: PHD Student
Location: Spitalgasse 4
Telephone: 40160 - 34091
E-Mail:
Principle Investigator(s):
Abstract:
X-linked adrenoleukodystrophy is a neurometabolic disorder caused by mutations in the ABCD1 gene encoding the peroxisomal half-transporter ABCD1. In its severest form, X-ALD characterized by progressive, inflammatory demyelination in the brain. The contribution of myelin-producing oligodendrocytes to the pathophysiology of X-ALD still remains largely unknown. We are conducting neuropathological analyses on human post mortem X-ALD tissue to gain insight into the status of oligodendrocytes within the demyelinative lesions. Furthermore, by utilizing the cuprizone-induced demyelination paradigm in Abcd1-deficient mice, we are able to parse out the processes of demyelination and remyelination in the context of the inherent oligodendrocytic metabolic disturbances.
Techniques:
· Transcardial perfusion
· Histology and immunohistochemistry
· Biochemical analyses (qPCR, Western Blot)
· RNA in situ hybridization
· Electron microscopy
Selected Publications: