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本研究采用微阵列（microarray）技术，检测溶酶体贮积症（lysosomal storage disease）的小鼠模型——单基因遗传性Ⅶ型黏多糖贮积症（mucopolysaccharidosis type VII, MPS VII）的不同脑区通路差异。本次分析发现多项意料之外的系统与进程改变：免疫系统上调但炎性改变极少，该特征与亲缘关系密切的MPS IIIb模型存在显著差异；尽管组织病理学未观察到白质病变，但主要少突胶质细胞（oligodendrocyte）基因的表达出现下调；同时存在大量发育相关基因的表达变化。本数据集共包含94份无重复样本，其中半数为野生型小鼠，半数为在C3H/HeOuJ遗传背景上回交得到的MPS VII突变型小鼠。