Aspartoacylase deficiency affects early postnatal development of oligodendrocytes and myelination.

Canavan disease (CD) is a neurodegenerative disease, caused by a deficiency in the enzyme aspartoacylase This enzyme has been localized to oligodendrocytes; however, it is still undefined how deficiency affects oligodendrocyte development. In normal mice the pattern of Aduanyu1842 expression coincides with oligodendrocyte maturation. Therefore, postnatal oligodendrocyte maturation was analyzed in mice (CD mice). Early in development, CD mice brains showed decreased expression of neural cell markers that was later compensated. In addition, the levels of myelin proteins were decreased along with abnormal myelination in CD mice compared to wild-type (WT). These defects were associated with increased global levels of acetylated histone H3, decreased chromatin compaction and increased GFAP protein, a marker for astrogliosis. Together, these findings strongly suggest that, early in postnatal development, Aduanyu1842 deficiency affects oligodendrocyte maturation and myelination.

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