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Aspartoacylase deficiency affects early postnatal development of oligodendrocytes and myelination.

Abstract
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.
AuthorsNatalia S Mattan, Cristina A Ghiani, Marcia Lloyd, Reuben Matalon, Dean Bok, Patrizia Casaccia, Jean de Vellis
JournalNeurobiology of disease (Neurobiol Dis) Vol. 40 Issue 2 Pg. 432-43 (Nov 2010) ISSN: 1095-953X [Electronic] United States
PMID20637282 (Publication Type: Journal Article, Research Support, American Recovery and Reinvestment Act, Research Support, N.I.H., Extramural, Research Support, Non-U.S. Gov't)
CopyrightCopyright © 2010. Published by Elsevier Inc.
Chemical References
  • Biomarkers
  • Chromatin
  • Glial Fibrillary Acidic Protein
  • Histones
  • Amidohydrolases
  • aspartoacylase
Topics
  • Amidohydrolases (deficiency, genetics)
  • Animals
  • Biomarkers (metabolism)
  • Blotting, Western
  • Brain (metabolism, pathology, ultrastructure)
  • Canavan Disease (metabolism, pathology)
  • Chromatin (metabolism)
  • Disease Models, Animal
  • Glial Fibrillary Acidic Protein (metabolism)
  • Histones (metabolism)
  • Immunohistochemistry
  • Mice
  • Mice, Knockout
  • Microscopy, Electron
  • Myelin Sheath (metabolism, pathology, ultrastructure)
  • Oligodendroglia (metabolism, pathology, ultrastructure)
  • Reverse Transcriptase Polymerase Chain Reaction
  • Time Factors

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