Inflammatory Neurodegeneration Caused by Inactivation of Peroxisome Function in Oligodendrocytes 

Dr. Celia Kassmann of the Department of Neurogenetics, Max-Planck-Institute of Experimental Medicine discussed her recent studies on inflammatory neurodegeneration caused by inactivation of peroxisome function in oligodendrocytes. 

X-linked adrenoleukodystrophy (X-ALD) is the most frequent juvenile leukodystrophy. It is caused by mutations of the adrenoleukodystrophy protein (ALDP), a peroxisomal membrane protein of unknown function. Attempts to create a mouse model for X-ALD by inactivating the ALDP gene have failed. Although ALDP deficient mice accumulate very long chain fatty acids (VLCFA) inflammatory brain demyelination does not occur. Dr. Kassmann investigated how peroxisomes in oligodendrocytes (the cells that form myelin in the brain) help maintain CNS myelin. Her laboratory generated mutant mouse that lacked functional peroxisomes only in oligodendrocytes. She found that peroxisomes in these cells are essetial for maintaining white matter (myelin) tracts. The mutant mice developed normally, but within several months exhibited ataxia, tremor, and premature death. They also showed widespread axonal degeneration, progressive subcortical demyelination, and a strong brain inflammation. The exact function of oligodendroglial peroxisomes is still unknown. But, Dr. Kassmann's studies suggest that functional peroxisomes are required for axonal support. 

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Celia Kassman, PhD

Source: The Myelin Project Annual Meeting - 2008

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