Investigation of loss of Cathepsin B in Niemann Pick disease, type C1
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资源简介：
Experiments investigating the effect of the loss of Cathepsin B (Ctsb-/-) in Niemann Pick type C1 deficient mice.
Summary: Double mutant mice with the loss of Cathepsin B (Ctsb) and Niemann Pick type C1 (Npc1) were generated and analyzed with respect weight, beam walk, disease severity score and survival. The results were compared to control mice as indicated.
Results: Double mutant mice (Ctsb-/-/Npc1-/-) exhibited a reduced weight gain pattern, a more severe balance and coordination assessment, a more severe disease burden and a reduced survival compared to control mutant mice (Ctsb+/-/Npc1-/-).
Conclusions: Lack of Cathepsin B in the NPC1 mutant mice increases the severity of the NPC1 disease.
创建时间：
2025-06-27
