Gene expression analysis of PiZ mouse livers

Transgenic PiZ mice have been genetically engineered to express ATZ and have been a valuable experimental model for studing liver disease associated with AAT deficiency. ATZ accumulates in these mice within the ER of hepatocytes in a nearly identical manner to livers of affected patients. To investigate the pathogenesis of liver damage induced by ATZ, we performed gene expression analysis in livers of 6-week-old PiZ mice and strain-, age-, and gender-matched wild-type mouse controls. All samples were processed on Affymetrix Mouse 430A 2.0 arrays using GeneChip 3’-IVT Plus and Hybridization Wash and Stain kits by means of Affymetrix’s standard protocols. The analysis indicated that most genes upregulated in PiZ livers were associated with response to unfolded proteins, ER nuclear signaling pathway, and response to protein stimulus. Gene expression analysis of 6 week-old wild-type (WT) and PiZ mouse livers

转基因PiZ小鼠经基因工程改造以表达ATZ，是研究与抗胰蛋白酶缺乏症（AAT deficiency）相关肝脏疾病的珍贵实验模型。ATZ会以与受累患者肝脏几乎一致的方式，在这些小鼠的肝细胞内质网（ER）中蓄积。为探究ATZ诱导肝损伤的发病机制，我们对6周龄PiZ小鼠，以及品系、年龄、性别匹配的野生型对照小鼠的肝脏开展了基因表达分析。所有样本均采用Affymetrix标准流程，使用GeneChip 3’-IVT Plus试剂盒与杂交洗涤染色试剂盒，在Affymetrix Mouse 430A 2.0基因芯片上完成处理。分析结果显示，PiZ小鼠肝脏中上调的多数基因与未折叠蛋白应答、内质网核信号通路及蛋白刺激应答相关。6周龄野生型（WT）与PiZ小鼠肝脏的基因表达分析