Journal
NEUROBIOLOGY OF DISEASE
Volume 40, Issue 2, Pages 432-443Publisher
ACADEMIC PRESS INC ELSEVIER SCIENCE
DOI: 10.1016/j.nbd.2010.07.003
Keywords
Oligodendrocytes; Neural cells; Development; Canavan disease; Aspartoacylase; Leukodystrophy
Categories
Funding
- NINDS
- NIH [P01-HD06576, HD04612, NMSS PP1585, RO1-NS42925-08, NS42925-S1]
- UCLA [EY00331]
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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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