Diseases

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.

National Library of MedicineMedical Subject Headings2026

Structured Summary

Abstract

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.

MeSH Record

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MeSH Record

Synonyms

5 entry terms
  • Proteinopathies, TDP-43
  • Proteinopathy, TDP-43
  • TDP 43 Proteinopathies
  • TDP-43 Proteinopathy
  • TDP-43-Mediated Neurodegeneration

MeSH Record

Aspects Covered

35 allowable subheadings

Indexed with the subheadings blood, cerebrospinal fluid, chemically induced, classification, complications, congenital, diagnosis, diagnostic imaging, diet therapy, drug therapy, economics, embryology, enzymology, epidemiology, ethnology, etiology, genetics, history, immunology, metabolism, microbiology, mortality, nursing, parasitology, pathology, physiopathology, prevention & control, psychology, radiotherapy, rehabilitation, surgery, therapy, urine, veterinary, virology.

MeSH Record

History Note

2010

MeSH Hierarchy

Tree Numbers

  1. C10w.574w.950
  2. C18.452w.845.800

AMA Style

References

  1. National Library of Medicine. TDP-43 Proteinopathies. Medical Subject Headings (MeSH). 2026. Unique ID D057177. http://id.nlm.nih.gov/mesh/2026/D057177
  2. TDP-43 Proteinopathies. In: Wikidata. https://www.wikidata.org/wiki/Q54920211