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TDP-43 Proteinopathies Summary

Description: 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: 129 relevant articles (3 outcomes, 13 trials/studies) for this Disease

Key Drugs and Agents for TDP-43 Proteinopathies

Efficacy Chart >>
Drugs and Important Biological Agents (IBA) related to treatments:
  1. protein TDP-43 (TDP-43) : 2 outcomes 8 studies in 92 results : IBA
  2. dimebolin (dimebon) : 1 outcome in 1 result : IBA
  3. Antibodies : 2 studies in 2 results : IBA
  4. Ubiquitin : 1 study in 16 results : IBA
  5. Proteasome Endopeptidase Complex (Proteasome) : 1 study in 2 results : IBA
  6. Insulin Receptor Substrate Proteins : 1 study in 1 result : IBA
  7. Antisense Oligonucleotides : 1 study in 1 result : IBA
  8. Serine (L-Serine) : 1 study in 1 result : FDA 16
  9. Messenger RNA (mRNA) : 1 study in 1 result : IBA
  10. Nucleic Acids : 1 study in 1 result : IBA
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Diseases Related to TDP-43 Proteinopathies

  1. Frontotemporal Lobar Degeneration (Semantic Dementia)
  2. Neurodegenerative Diseases (Neurodegenerative Disease)
  3. Tauopathies
  4. Cystic Fibrosis (Mucoviscidosis)
  5. Amyotrophic Lateral Sclerosis (Lou Gehrig's Disease)
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Key Therapies for TDP-43 Proteinopathies

Efficacy Chart >>
  1. Therapeutics : 1 study in 2 results
  2. Lasers (Laser) : 1 study in 1 result

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