Characterization of AEP/Legumain-deficient mice as a new model of lysosome disease

天冬酰胺内肽酶（AEP）是一种溶酶体半胱氨酸蛋白酶。我们已经将aep缺陷小鼠描述为人类吞噬细胞综合征的疾病模型，其发展为噬血细胞症，特别是在骨髓中。戈谢病是溶酶体贮积病的一种，部分噬血细胞与戈谢病相似。我们进行了进一步的检查，以表征aep缺陷小鼠作为溶酶体疾病的新模型。

Asparagine endopeptidase(AEP) is a lysosomal cysteine protease. We have characterized AEP-deficient mice as a disease model of human hemomphagocytic syndrome, which developed hemophagocytosis especially in bone marrow. A part of hemophagpocytes resembled Gaucher cells in Gaucher disease, one of lysosomal storage disease. We have conducted further examinations so as to characterize AEP-deficient mice as a new model of lysosomal disease.