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TDP-43 Proteinopathies

Diseases characterized by the presence of abnormally phosphorylated, ubiquitinated, and cleaved DNA-binding protein TDP-43 in affected brain and spinal cord. Inclusions of the pathologic protein in neurons and glia, without the presence of AMYLOID, is the major feature of these conditions, thus making these proteinopathies distinct from most other neurogenerative disorders in which protein misfolding leads to brain amyloidosis. Both frontotemporal lobar degeneration and AMYOTROPHIC LATERAL SCLEROSIS exhibit this common method of pathogenesis and thus they may represent two extremes of a continuous clinicopathological spectrum of one disease.
Also Known As:
Proteinopathies, TDP-43; Proteinopathy, TDP-43; TDP 43 Proteinopathies; TDP-43 Proteinopathy
Networked: 211 relevant articles (8 outcomes, 26 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1. Frontotemporal Lobar Degeneration (Degeneration, Frontotemporal Lobar)
2. Amyotrophic Lateral Sclerosis (Lou Gehrig's Disease)
3. Frontotemporal Dementia (Semantic Dementia)
4. Tauopathies
5. Synucleinopathies

Experts

1. Hasegawa, Masato: 15 articles (01/2022 - 11/2008)
2. Trojanowski, John Q: 14 articles (01/2018 - 07/2007)
3. Arai, Tetsuaki: 11 articles (10/2020 - 11/2008)
4. Akiyama, Haruhiko: 10 articles (10/2020 - 11/2008)
5. Lee, Virginia M-Y: 10 articles (01/2018 - 07/2007)
6. Zarnescu, Daniela C: 7 articles (01/2022 - 09/2015)
7. Yoshida, Mari: 7 articles (01/2022 - 11/2008)
8. Nonaka, Takashi: 7 articles (10/2020 - 11/2008)
9. Bigio, Eileen H: 7 articles (11/2017 - 07/2007)
10. Nelson, Peter T: 6 articles (01/2022 - 01/2019)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to TDP-43 Proteinopathies:
1. Proteins (Proteins, Gene)FDA Link
2. VaccinesIBA
3. EdaravoneIBA
4. Long Noncoding RNAIBA
5. Neuroprotective AgentsIBA
6. Cholinergic Agents (Cholinergics)IBA
7. Fingolimod Hydrochloride (FTY720)FDA Link
8. latrepirdine (dimebon)IBA
9. RNA (Ribonucleic Acid)IBA
10. ProgranulinsIBA

Therapies and Procedures

1. Therapeutics
2. Lasers (Laser)
3. Immunotherapy
4. Oral Administration
5. Activities of Daily Living (ADL)