Expression data of Normal versus Mutant MPS VII C3H mouse

We used microarray to detect pathway differences in the various brain regions in a monogenic in mucopolysaccharidosis type VII ( MPS VII ), a mouse model of a lysosomal storage disease A number of changes revealed unexpected system and process alterations, such as upregulation of the immune system with few inflammatory changes (a significant difference from the closely related MPS IIIb model), down-regulation of major oligodendrocyte genes even though white matter changes are not a feature histopathologically, and a plethora of developmental gene changes. 94 samples, no replicates, made up of half normals and half MPS mutant mice for the MPS VII mutation backcrossed on a C3h-heouj background

本研究以溶酶体贮积症（lysosomal storage disease）的小鼠模型——单基因型黏多糖贮积症VII型（mucopolysaccharidosis type VII, MPS VII）——为研究对象，采用微阵列（microarray）技术检测该模型不同脑区的通路差异。实验中观察到诸多意料之外的系统与细胞进程异常：其一，免疫系统通路出现上调，但炎症相关变化却较为有限，这与亲缘关系密切的MPS IIIb模型存在显著差异；其二，尽管组织病理学层面未发现白质病变特征，但主要少突胶质细胞（oligodendrocyte）基因的表达却呈现下调；此外，还存在大量发育相关基因的表达改变。本数据集共包含94份样本，无生物学重复样本，其中一半为野生型对照小鼠，另一半为在C3H-HeOuJ背景上回交得到的MPS VII突变型小鼠。