Aspartoacylase deficiency affects early postnatal development of oligodendrocytes and myelination.

摘要: Canavan disease (CD) is a neurodegenerative disease, caused by deficiency in the enzyme aspartoacylase (ASPA). This has been localized to oligodendrocytes; however, it still undefined how ASPA affects oligodendrocyte development. In normal mice pattern of expression coincides with maturation. Therefore, postnatal maturation was analyzed ASPA-deficient (CD mice). Early development, CD brains showed decreased neural cell markers that later compensated. addition, levels myelin proteins were along abnormal myelination compared wild-type (WT). These defects associated increased global acetylated histone H3, chromatin compaction and GFAP protein, marker for astrogliosis. Together, these findings strongly suggest that, early myelination.