期刊
NEUROBIOLOGY OF DISEASE
卷 40, 期 2, 页码 432-443出版社
ACADEMIC PRESS INC ELSEVIER SCIENCE
DOI: 10.1016/j.nbd.2010.07.003
关键词
Oligodendrocytes; Neural cells; Development; Canavan disease; Aspartoacylase; Leukodystrophy
资金
- NINDS
- NIH [P01-HD06576, HD04612, NMSS PP1585, RO1-NS42925-08, NS42925-S1]
- UCLA [EY00331]
Canavan disease (CD) is a neurodegenerative disease, caused by a deficiency in the enzyme aspartoacylase (ASPA). This enzyme has been localized to oligodendrocytes; however, it is still undefined how ASPA deficiency affects oligodendrocyte development. In normal mice the pattern of ASPA expression coincides with oligodendrocyte maturation. Therefore, postnatal oligodendrocyte maturation was analyzed in ASPA-deficient 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, ASPA deficiency affects oligodendrocyte maturation and myelination. (c) 2010 Published by Elsevier Inc.
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