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.

Year introduced: 2010

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Tree Number(s): C10.574.950, C18.452.845.800

MeSH Unique ID: D057177

Entry Terms:

• Proteinopathies, TDP-43
• Proteinopathy, TDP-43
• TDP 43 Proteinopathies
• TDP-43 Proteinopathy

All MeSH Categories
Diseases Category
Nervous System Diseases
Neurodegenerative Diseases
TDP-43 Proteinopathies
Amyotrophic Lateral Sclerosis
Frontotemporal Lobar Degeneration
Frontotemporal Dementia
Primary Progressive Nonfluent Aphasia

All MeSH Categories
Diseases Category
Nutritional and Metabolic Diseases
Metabolic Diseases
Proteostasis Deficiencies
TDP-43 Proteinopathies
Amyotrophic Lateral Sclerosis
Frontotemporal Lobar Degeneration
Frontotemporal Dementia
Primary Progressive Nonfluent Aphasia