[Abstract Title]. - Society for Neuroscience

nodes were collected at different points in the disease course in order to compare cytokine
production. Luminex assay at 38 days post-immunization showed that E2 treated animals had
dramatically reduced secretion of IL-17 in cells isolated from brain compared to control animals.
Production from splenocytes also showed a decrease, but IL-17 levels were unchanged in the
lymph node. The effects of IL-23 and the role of the receptors ER-α and ER-β are also being
explored.
Disclosures: M.A. Yates, None; S. Sinha, None; L.J. Kaler, None; H. Offner, Effective
Pharmaceuticals, E. Ownership Interest (stock, stock options, patent or other intellectual
property).
Poster
251. Demyelinating Disorders: Animal Models and Human Studies I
Time: Sunday, November 16, 2008, 1:00 pm - 5:00 pm
Program#/Poster#: 251.27/W20
Topic: C.08.b. Animal models
Support: NIH NINDS Grant 5F31NS056502
Title: Effect of ASPA deficiency on neural cells during early postnatal development
Authors: *N. S. MATTAN 1 , R. MATALON 2 , J. DE VELLIS 1 ;
1 Dept Neurobiol, UCLA MRRC, Los Angeles, CA; 2 UTMB - Dept. of Pediatrics, Galveston, TX
Abstract: Canavan Disease (CD) is a neurodegenerative disease, caused by a deficiency in the
enzyme aspartoacylase (ASPA), and characterized by mental retardation, macrocephaly, head lag
and hypotonia. Loss of ASPA activity causes a consequent increase in its substrate Nacetylaspartate
(NAA) and decreased production of aspartate and acetate. Recently, ASPA has
been localized in oligodendrocytes (OL), however, it is still undefined how ASPA deficiency
may affect OL development. To investigate the effects of ASPA deficiency on the development
of OL cells, we studied postnatal OL maturation in ASPA CD and wild-type (WT) mice at
different ages. ASPA levels in WT mice had an expression pattern that correlates well with OL
lineage progression and its expression peaked at postnatal day 30 (P30) in different brain regions
suggesting a role for ASPA in OL development and myelination. CD and WT mice showed
similar number of Sox2 and Sox 9 positive cells as well as immature OL at early postnatal ages
(P1 and P7). However, more progenitor cells expressing Sox2 were found up to P30 in CD brains
opposed to WT mice. Defects in OL maturation and myelinogenesis started to be seen in CD
brains as early as P10 and P15. Decreased MBP and PLP immunoreactivity was found in both
the corpus callosum (CC) and the CB of CD mice. MBP protein levels were decreased by P15 in