Peroxisomal dysfunction and ER stress -A novel mechanism for the demyelination in central nervous system-

The purpose of this project was to evaluate the relationship peroxisomal dysfunction anddemyelination. In this study, we focused on the ER stress and innate immunity in ABCD1-deficient primary astrocytes. Dysfunction of ABCD1, a peroxisomal membrane protein, caused a neurodegenerative disease called adrenoleukodystropy. We found that treatment of ligands for pattern recognition receptors induced the expression of cytokines and antiviral proteins in ABCD1-deficient astrocytes. These results suggest that dysfunction of ABCD1 lead to the disturbance of innate immune response, which result in the demyelination in adrenoleukodystrophy.