Functional alteration of astrocytes induced by GFAP mutation and the modified factors

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Alexander disease is a rare neurodegenerative disease caused by the mutations encoding glial fibrillary acidic protein(GFAP). To identify modified factors to clarify functional alteration of glial cells with mutant GFAP, we performed microarray analysis, real-time PCR or GFAP promoter gene analysis using cell models or patient's DNA. These studies indicated αB-crystallin may play an important role in mutant GFAP cells and the alteration of GFAP expression controlled by GFAP promoter gene may modify the clinical course. We also prepared Drosophia model constructed by inducing GFAP gene as a new disease model.