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.

Descriptor ID	D057177
MeSH Number(s)	C10.574.950 C18.452.845.800
Concept/Terms	TDP-43 Proteinopathies TDP-43 Proteinopathies Proteinopathies, TDP-43 Proteinopathy, TDP-43 TDP 43 Proteinopathies TDP-43 Proteinopathy

Below are MeSH descriptors whose meaning is more general than "TDP-43 Proteinopathies".

Below are MeSH descriptors whose meaning is related to "TDP-43 Proteinopathies".

Below are MeSH descriptors whose meaning is more specific than "TDP-43 Proteinopathies".