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Prion-like properties of pathological TDP-43 aggregates from diseased brains
Nonaka T, Masuda-Suzukake M, Arai T, Hasegawa Y, Akatsu H, Obi T, Yoshida M, Murayama S et al.. Cell reports, 2013-07-03
Topic: Brain proteins, microglia and lab models
TDP-43 is the major component protein of ubiquitin-positive inclusions in brains of patients with frontotemporal lobar degeneration (FTLD-TDP) or amyotrophic lateral sclerosis (ALS). Here, we report the characterization of prion-like properties of aggregated TDP-43 prepared from diseased brains. When insoluble TDP-43 from ALS or FTLD-TDP brains was introduced as seeds into SH-SY5Y cells expressing TDP-43, phosphorylated and ubiquitinated TDP-43 was aggregated in a self-templating manner. Immunoblot analyses revealed that the C-terminal fragments of insoluble TDP-43 characteristic of each disease type acted as seeds, inducing seed-dependent aggregation of TDP-43 in these cells. The seeding ability of insoluble TDP-43 was unaffected by proteinase treatment but was abrogated by formic acid. One subtype of TDP-43 aggregate was resistant to boiling treatment. The insoluble fraction from cells harboring TDP-43 aggregates could also trigger intracellular TDP-43 aggregation. These results indicate that insoluble TDP-43 has prion-like properties that may play a role in the progression of TDP-43 proteinopathy.
Cite this study APA, Vancouver, BibTeX · download .bib or .ris
APA 7th
Nonaka, T., Masuda-Suzukake, M., Arai, T., Hasegawa, Y., Akatsu, H., Obi, T., et al. (2013). Prion-like properties of pathological TDP-43 aggregates from diseased brains. Cell reports. https://doi.org/10.1016/j.celrep.2013.06.007
Vancouver
Nonaka T, Masuda-Suzukake M, Arai T, Hasegawa Y, Akatsu H, Obi T, et al. Prion-like properties of pathological TDP-43 aggregates from diseased brains. Cell reports. 2013. doi:10.1016/j.celrep.2013.06.007. PMID: 23831027.
BibTeX
@article{nonaka2013-23831027,
author = {Nonaka, T. and Masuda-Suzukake, M. and Arai, T. and Hasegawa, Y. and Akatsu, H. and Obi, T. and Yoshida, M. and Murayama, S. and others},
title = {{Prion-like properties of pathological TDP-43 aggregates from diseased brains}},
journal = {Cell reports},
year = {2013},
doi = {10.1016/j.celrep.2013.06.007},
pmid = {23831027},
url = {https://doi.org/10.1016/j.celrep.2013.06.007}
}
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Key findings
- Immunoblot analyses revealed that the C-terminal fragments of insoluble TDP-43 characteristic of each disease type acted as seeds, inducing seed-dependent aggregation of TDP-43 in these cells.
- These results indicate that insoluble TDP-43 has prion-like properties that may play a role in the progression of TDP-43 proteinopathy.
Why it matters
TDP-43 clumps taken from the brains of people with ALS or frontotemporal lobar degeneration (FTLD-TDP) made TDP-43 clump in cultured cells "in a self-templating manner", and the clumps formed in those cells could seed the process again. The seeding fragments were "characteristic of each disease type", and one subtype survived boiling. The authors conclude TDP-43 has prion-like properties that "may play a role" in disease progression. This was a cell-culture study of ALS/FTLD brain material, not of Alzheimer's, and it tested no treatment.
Study details
- Evidence strengthLow
How this study scored 51/100
Published in a peer-reviewed journal. Scored zero for study design, sample size, effect reported, studied in people and randomized / blinded because the record does not state it.
| Component | Points | Why |
|---|---|---|
| Journal tier | 7 / 10 | Tier 3 — peer-reviewed journal (Cell reports) |
| Journal's citation record | 3 / 4 | Cell Reports: journal h-index 321 (OpenAlex, 2026-10-29) |
| Study design | 0 / 12 | No study design recorded — scores zero, not guessed (not stated in the record — scored 0) |
| Strength of evidence | 1 / 5 | Rated “low” in our summary |
| Peer-review validation | 10 / 10 | Peer-reviewed. Published as an article in a named peer-reviewed journal. |
| How recent | 1 / 5 | 2013 — within 20 year(s) of the newest study here |
| Sample size | 0 / 4 | Participant count not stated — scores zero (not stated in the record — scored 0) |
| Effect reported | 0 / 3 | No effect size reported — scores zero (not stated in the record — scored 0) |
| Studied in people | 0 / 3 | Not stated whether this was studied in people — scores zero (not stated in the record — scored 0) |
| Randomized / blinded | 0 / 2 | No randomization or blinding stated — scores zero (not stated in the record — scored 0) |
| Times cited | 6 / 7 | Cited 521 times (OpenAlex, 2026-10-10) |
| Citations per year | 4 / 5 | 34.7 citations a year over 15 year(s) since 2013 |
| Impact for its field and year | 5 / 6 | Cited 23.4× the average for papers of its field and year (OpenAlex FWCI) |
| Cited by studies here | 3 / 6 | Cited by 19 other studies in this library |
| Still being cited | 3 / 4 | Cited 90 times in 2024–2026 (2026 still in progress; OpenAlex) |
| Senior author's track record | 3 / 4 | Last-listed author Masato Hasegawa: h-index 97 (OpenAlex, whole career) |
| Institution's track record | 1 / 3 | University of Manchester: institution h-index 1,304 (OpenAlex) |
| Multicenter collaboration | 2 / 3 | OpenAlex lists 7 institution(s) in 2 countries |
| Indexed in PubMed | 2 / 2 | Indexed in PubMed (PMID 23831027) |
| Free to read | 0 / 2 | Behind a paywall — scores zero |
| Total | 51 / 100 | — |
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