Expression data of the thymus from 15weeks old Hexb-/- and Hexb+/- mice

Sandhoff disease (SD) is a lysosomal storage disorder characterized by the absence of β-hexosaminidase and storage of GM2 ganglioside and related glycolipids. We found the alterations in the thymus during the development of mild to severe progressive neurologic disease. To elucidate the molecular basis of thymic involution, we performed cDNA microarray analysis to identify the changes in gene expression that accompanied the involution of the thymus. 8018 probes were found to be relatively increased in the thymus of the Hexb-/- mice, an animal model for SD, compared with that of the Hexb+/- mice. On the other hand, the expression of 7604 probes was relatively decreased. The cohort of up-regulated sequences was dominated by genes that play a role in the immune response. In addition, some of these genes are expressed in macrophage lineages such as macrophage expressed gene 1, and colony stimulating factor 2 receptor beta 1. Th2 cytokines were mostly upregulated in the Hexb-/- mice, although Th1 cytokines did not show this increase. In addition, B cell related genes such as CD19, CXCL13 were increased, whereas the T cell-related genes were mostly decreased, in Hexb-/- mice compared with Hexb+/- mice. Pooled thymic RNA samples were obtained from two 15 weeks old Hexb-/- mice and two strain-matched, 15 weeks old Hexb+/- mice. cDNA were synthesized from 10 μg total RNA. Biotinylated cRNA were synthesized. Following fragmentation, 10 μg of cRNA were hybridized on GeneChip Mouse Genome 430 2.0 Array.