Evaluation of the Potential Role of Proprotein Convertase Subtilisin/Kexin Type 9 (PCSK9) in Niemann–Pick Disease, Type C1
Abstract
:1. Introduction
2. Results
2.1. Knockout of Pcsk9 Did not Significantly Alter Weight Progression in Affected Npc1-/- Mice
2.2. NPC1 Neurological Disease Progression Was Not Affected by Disruption of Pcsk9
2.3. Lifespan of Npc1-/-/Pcsk9-/- Mice is not Significantly Altered
2.4. Cerebellar Analysis of Purkinje Neurons by Immunohistochemical Staining
2.5. Serum Liver Enzymes and Cholesterol Analyses
3. Discussion
4. Materials and Methods
4.1. Animal Maintenance
4.2. Disease Severity Scoring
4.3. AccuScan Activity Assessment
4.4. Immunohistochemical Analysis of Cerebellar Tissue from Mutant Mice
4.5. Serum Liver Enzymes and Cholesterol Analyses
4.6. Statistical Analysis
Supplementary Materials
Author Contributions
Funding
Acknowledgments
Conflicts of Interest
Abbreviations
NPC1 | Niemann–Pick disease, type C1 |
PCSK9 | Proprotein converting subtilisin/Kexin 9 |
LDLR | Low density lipoprotein receptor |
VLDLRApoER2 | Very low-density lipoprotein receptorApolipoprotein E receptor 2 |
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Cawley, N.X.; Lyons, A.T.; Abebe, D.; Wassif, C.A.; Porter, F.D. Evaluation of the Potential Role of Proprotein Convertase Subtilisin/Kexin Type 9 (PCSK9) in Niemann–Pick Disease, Type C1. Int. J. Mol. Sci. 2020, 21, 2430. https://doi.org/10.3390/ijms21072430
Cawley NX, Lyons AT, Abebe D, Wassif CA, Porter FD. Evaluation of the Potential Role of Proprotein Convertase Subtilisin/Kexin Type 9 (PCSK9) in Niemann–Pick Disease, Type C1. International Journal of Molecular Sciences. 2020; 21(7):2430. https://doi.org/10.3390/ijms21072430
Chicago/Turabian StyleCawley, Niamh X., Anna T. Lyons, Daniel Abebe, Christopher A. Wassif, and Forbes D. Porter. 2020. "Evaluation of the Potential Role of Proprotein Convertase Subtilisin/Kexin Type 9 (PCSK9) in Niemann–Pick Disease, Type C1" International Journal of Molecular Sciences 21, no. 7: 2430. https://doi.org/10.3390/ijms21072430