Postmortem Quantitative Analysis of Prion Seeding Activity in the Digestive System
Abstract
:1. Introduction
2. Results
2.1. Analysis of Extraneural Tissues from Patients with sCJD and Healthy Subjects Using Endpoint RT-QuIC Assay
2.2. Analysis of Extraneural Tissues from a Patient with GSS and a Patient with Genetic HPD Using Endpoint RT-QuIC Assay
2.3. Immunohistochemical Staining of the Gut
3. Discussion
4. Materials and Methods
4.1. Patients
4.2. Tissue Homogenate Preparation
4.3. Endpoint RT-QuIC
5. Conclusions
Author Contributions
Funding
Acknowledgments
Conflicts of Interest
References
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Sample Availability: Samples of the compounds are not available from the authors. |
Tissue | Patient 1 | Patient 2 | Patient 3 | Patient 4 | ||||
---|---|---|---|---|---|---|---|---|
Mean #3 | ±S.D. | Mean | ±S.D. | Mean | ±S.D. | Mean | ±S.D. | |
Esophagus | ≤6.70 | 8.38 | ±0.16 | 7.98 | ±0.39 | N.E. | ||
Stomach | ≤6.50 | 7.10 | ±0.14 | ≤6.57 | ≤6.80 | |||
Duodenum | N.E | 7.10 | ±0.70 | 6.24 | ±0.25 | 8.31 | ±0.20 | |
Jejunum | ≤6.67 | 7.20 | ±0.12 | 6.44 | ±0.10 | ≤6.67 | ||
Terminal ileum | 7.07 | ±0.26 | 7.74 | ±0.25 | ≤6.90 | N.D. | ||
Transverse colon | N.E. | ≤6.14 | ≤7.00 | 6.50 | ±0.11 | |||
Sigmoid colon | ≤6.12 | N.D. | N.E. | 7.60 | ±0.17 | |||
Brain #3 | 10.08 | ±0.12 | 9.42 | ±0.12 | 9.17 | ±0.42 | 10.00 | ±0.35 |
Tissue | Patient 5 | |
---|---|---|
Mean | ±S.D. | |
Esophagus | 6.93 | ±0.32 |
Stomach | 7.34 | ±0.20 |
Duodenum | ≤6.57 | |
Jejunum | 7.22 | ±0.30 |
Appendix | 8.33 | ±0.67 |
Transverse colon | 8.46 | ±0.44 |
Sigmoid colon | N.E. | |
Brain | 10.18 | ±0.34 |
Tissue | Patient 6 | |
---|---|---|
Mean | ±S.D. | |
Esophagus | 8.5 | ±0.00 |
Stomach | 8.21 | ±0.34 |
Duodenum | 6.81 | ±0.31 |
Jejunum | ≤6.33 | ±0.00 |
Ileum | 7.17 | ±0.94 |
Appendix | 7.23 | ±0.20 |
Cecum | ≤6.64 | |
Transverse colon | ≤5.80 | |
Sigmoid colon | 7.47 | ±0.50 |
Brain | 10.3 | ±0.35 |
Patient 1 | Patient 2 | Patient 3 | Patient 4 | Patient 5 | Patient 6 | |
---|---|---|---|---|---|---|
Sporadic CJD | Sporadic CJD | Sporadic CJD | Sporadic CJD | GSS (P102L) | Genetic CJD (E200K) | |
sex | male | male | female | male | male | male |
codon 129 | MV | MM | MM | MM | MM | MM |
Typing of PrP-res | type 2 | type 1 | type 1 | type 1 | - | type 1 |
Age at onset (years) | 69 | 70 | 59 | 62 | 43 | 66 |
Period from onset to death (months) | 27 | 18 | 78 | 60 | 39 | 6 |
Period from onset to akinetic mutism (months) | 3 | 2 | 3 | 4 | 25 | 3 |
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Share and Cite
Satoh, K.; Fuse, T.; Nonaka, T.; Dong, T.; Takao, M.; Nakagaki, T.; Ishibashi, D.; Taguchi, Y.; Mihara, B.; Iwasaki, Y.; et al. Postmortem Quantitative Analysis of Prion Seeding Activity in the Digestive System. Molecules 2019, 24, 4601. https://doi.org/10.3390/molecules24244601
Satoh K, Fuse T, Nonaka T, Dong T, Takao M, Nakagaki T, Ishibashi D, Taguchi Y, Mihara B, Iwasaki Y, et al. Postmortem Quantitative Analysis of Prion Seeding Activity in the Digestive System. Molecules. 2019; 24(24):4601. https://doi.org/10.3390/molecules24244601
Chicago/Turabian StyleSatoh, Katsuya, Takayuki Fuse, Toshiaki Nonaka, Trong Dong, Masaki Takao, Takehiro Nakagaki, Daisuke Ishibashi, Yuzuru Taguchi, Ban Mihara, Yasushi Iwasaki, and et al. 2019. "Postmortem Quantitative Analysis of Prion Seeding Activity in the Digestive System" Molecules 24, no. 24: 4601. https://doi.org/10.3390/molecules24244601