The DOI matches its Crossref record; 45 of the 45 references it cites were found in a registry or an index.
Prion-like Properties of Pathological TDP-43 Aggregates from Diseased Brains
Open accessAbstract
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.
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Molecular Mechanisms of TDP-43 Misfolding and Pathology in Amyotrophic Lateral Sclerosis
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ALS Genetics, Mechanisms, and Therapeutics: Where Are We Now?
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TDP-43 repression of nonconserved cryptic exons is compromised in ALS-FTD
Ling, J. P., Pletnikova, O., Troncoso, J. C., & Wong, P. C. (2015) · Science · doi:10.1126/science.aab0983 · Cited by 688
Metadata
- Authors
- Nonaka, T., Masuda-Suzukake, M., Arai, T., Hasegawa, Y., Akatsu, H., Obi, T., Yoshida, M., Murayama, S., Mann, D. M. A., Akiyama, H., & Hasegawa, M.
- Venue
- Cell Reports
- Publisher
- Elsevier BV
- Published
- 2013-07
- Volume
- 4
- Issue
- 1
- Pages
- 124-134
- ISSN
- 2211-1247 (ISSN-L)
- OpenAlex
- W2170087857
- Cited by
- 521
- References
- 45
- FWCI
- 23.37
- Type
- journal article
- Language
- en
- Open access
- gold
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- Nonaka, T., Masuda-Suzukake, M., Arai, T., Hasegawa, Y., Akatsu, H., Obi, T., Yoshida, M., Murayama, S., Mann, D. M. A., Akiyama, H., & Hasegawa, M. (2013). Prion-like Properties of Pathological TDP-43 Aggregates from Diseased Brains. Cell Reports. https://doi.org/10.1016/j.celrep.2013.06.007
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