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

Nervous System Diseases: 14178
- Neurodegenerative Diseases: 42472
  - TDP-43 Proteinopathies: 211
Nutritional and Metabolic Diseases: 15
- Metabolic Diseases: 10142
  - Proteostasis Deficiencies: 1400
    - TDP-43 Proteinopathies: 211

Chandra Pidathala Selected Research

Chandra Pidathala Research Topics

Disease

Drug/Important Bio-Agent (IBA)