Increased Regenerative Capacity of the Olfactory Epithelium in Niemann–Pick Disease Type C1
Abstract
:1. Introduction
2. Results
2.1. Histology of the Olfactory Mucosa
2.2. Quantification of BrdU(+) Proliferating Cells
2.3. Quantification of OMP(+) Mature ORNs
2.4. Quantification of Cas-3(+) Apoptotic Cells
2.5. Cathepsin D
3. Discussion
3.1. Olfactory Epithelium in NPC1+/+ Control Animals
3.2. Morphological Alterations in NPC1−/− Animals
3.3. Combination Treatment of NPC1−/− Mice Shows Beneficial Effects
3.4. Monotherapy with HPβCD
3.5. Limitations of Symptomatic Treatments
3.6. Therapy-Induced Changes in NPC1+/+ Mice
3.7. Differences between the Therapeutic Effects
4. Methods
4.1. Animals
4.2. Genotyping
4.3. Pharmacologic Treatment
4.4. BrdU Injections
4.5. Sample Preparation
4.6. Immunohistochemistry
4.7. Stereology and Statistic Evaluation
5. Conclusions
Acknowledgments
Author Contributions
Conflicts of Interest
References
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Treatment Group | BrdU (Cells/mm3 ± SEM) | OMP (Cells/mm3 ± SEM) | Cas-3 (Cells/mm3 ± SEM) |
---|---|---|---|
untreated NPC1+/+ | 17,417 ± 1317 | 836,392 ± 63,784 | 3999 ± 596 |
untreated NPC1−/− | 25,180 ± 1605 | 346,129 ± 37,812 | 9879 ± 1373 |
COMBI NPC1+/+ | 67,972 ± 7694 | 844,819 ± 65,313 | 3327 ± 387 |
COMBI NPC1−/− | 44,693 ± 4191 | 709,729 ± 69,558 | 5471 ± 397 |
HPΒCD NPC1+/+ | 75,871 ± 1865 | 797,470 ± 32,568 | 4316 ± 215 |
HPΒCD NPC1−/− | 70,558 ± 8159 | 584,268 ± 21,134 | 7016 ± 591 |
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Meyer, A.; Wree, A.; Günther, R.; Holzmann, C.; Schmitt, O.; Rolfs, A.; Witt, M. Increased Regenerative Capacity of the Olfactory Epithelium in Niemann–Pick Disease Type C1. Int. J. Mol. Sci. 2017, 18, 777. https://doi.org/10.3390/ijms18040777
Meyer A, Wree A, Günther R, Holzmann C, Schmitt O, Rolfs A, Witt M. Increased Regenerative Capacity of the Olfactory Epithelium in Niemann–Pick Disease Type C1. International Journal of Molecular Sciences. 2017; 18(4):777. https://doi.org/10.3390/ijms18040777
Chicago/Turabian StyleMeyer, Anja, Andreas Wree, René Günther, Carsten Holzmann, Oliver Schmitt, Arndt Rolfs, and Martin Witt. 2017. "Increased Regenerative Capacity of the Olfactory Epithelium in Niemann–Pick Disease Type C1" International Journal of Molecular Sciences 18, no. 4: 777. https://doi.org/10.3390/ijms18040777