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Descriptor English: TDP-43 Proteinopathies
Descriptor Spanish: Proteinopatías TDP-43
Descriptor proteinopatías TDP-43
Scope note: Enfermedades caracterizadas por la presencia de proteína TDP-43 de unión a ADN anormalmente dividida, fosforilada y ubicuitinada en el encéfalo y médula espinal afectados. Las inclusiones de la proteína patológica en las  neuronas y células gliales, sin la presencia de AMILOIDE, son la característica principal de estas afecciones, y distingue a estas proteinopatías de la mayoría de otros trastornos neurodegenerativos en los que los errores en el plegamiento de las proteínas conducen a amiloidosis cerebral. Tanto la degeneración frontotemporal como la ESCLEROSIS LATERAL AMIOTRÓFICA comparten este tipo de patogénesis y, por lo tanto, pueden representar los dos extremos de un espectro clínico-patológico continuo de una única enfermedad. 
Descriptor Portuguese: Proteinopatias TDP-43
Descriptor French: Protéinopathies TDP-43
Entry term(s): Proteinopathies, TDP-43
Proteinopathy, TDP-43
TDP 43 Proteinopathies
TDP-43 Proteinopathy
Tree number(s): C10.574.950
C18.452.845.800
RDF Unique Identifier: https://id.nlm.nih.gov/mesh/D057177
Scope note: 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.
Allowable Qualifiers: BL blood
CF cerebrospinal fluid
CI chemically induced
CL classification
CN congenital
CO complications
DG diagnostic imaging
DH diet therapy
DI diagnosis
DT drug therapy
EC economics
EH ethnology
EM embryology
EN enzymology
EP epidemiology
ET etiology
GE genetics
HI history
IM immunology
ME metabolism
MI microbiology
MO mortality
NU nursing
PA pathology
PC prevention & control
PP physiopathology
PS parasitology
PX psychology
RH rehabilitation
RT radiotherapy
SU surgery
TH therapy
UR urine
VE veterinary
VI virology
Public MeSH Note: 2010
History Note: 2010
DeCS ID: 53517
Unique ID: D057177
Documents indexed in the Virtual Health Library (VHL): Click here to access the VHL documents
Date Established: 2010/01/01
Date of Entry: 2009/07/06
TDP-43 Proteinopathies - Preferred
Concept UI M0534988
Scope note 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.
Preferred term TDP-43 Proteinopathies
Entry term(s) Proteinopathies, TDP-43
Proteinopathy, TDP-43
TDP 43 Proteinopathies
TDP-43 Proteinopathy



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