2026-10-15T08:50:04Z https://data.mendeley.com/oai

oai:data.mendeley.com/h2bvr7ggxr.1 2025-06-27T12:17:08Z

Cawley, Niamh Investigation of loss of Cathepsin B in Niemann Pick disease, type C1 Mendeley Data 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. Neurodegenerative Disorder Cholesterol Lysosomal Storage Disease Dataset doi:10.17632/h2bvr7ggxr.1 oai:data.mendeley.com/h2bvr7ggxr.1 Creative Commons Attribution 4.0 International http://creativecommons.org/licenses/by/4.0 https://data.mendeley.com/datasets/h2bvr7ggxr 2025-06-27T12:17:08Z