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Aspartoacylase deficiency affects early postnatal development of oligodendrocytes and myelination.

Neurobiol Dis.. 2010-11;  40(2):432-43
Mattan NS, Ghiani CA, Lloyd M, Matalon R, Bok D, Casaccia P, de Vellis J. a Department of Neurobiology, David Geffen School of Medicine, University of California Los Angeles, Los Angeles, CA 90095, USAb Department of Psychiatry, David Geffen School of Medicine, University of California Los Angeles, Los Angeles, CA 90095, USAc Intellectual and Developmental Disabilities Research Center, David Geffen School of Medicine, University of California Los Angeles, Los Angeles, CA 90095, USAd Jules Stein Eye Institute, David Geffen School of Medicine, University of California L
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摘要

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 inc... More

关键词

Oligodendrocytes; Neural cells; Development; Canavan disease; Aspartoacylase; Leukodystrophy