Motor neuron atrophy
|
0.100 |
Biomarker
|
disease |
BEFREE |
This study provided compelling evidence that ErbB4 is also involved in the pathophysiology of SALS, and that the disruption of the NRG-ErbB4 pathway may underlie the TDP-43-dependent motor neuron degeneration in ALS.
|
31124187 |
2019 |
Motor neuron atrophy
|
0.100 |
GeneticVariation
|
disease |
BEFREE |
Sarm1 deletion suppresses TDP-43-linked motor neuron degeneration and cortical spine loss.
|
31661035 |
2019 |
Motor neuron atrophy
|
0.100 |
GeneticVariation
|
disease |
BEFREE |
Mutant TDP-43 Causes Early-Stage Dose-Dependent Motor Neuron Degeneration in a TARDBP Knockin Mouse Model of ALS.
|
30625319 |
2019 |
Motor neuron atrophy
|
0.100 |
Biomarker
|
disease |
BEFREE |
Immunohistochemical examination confirmed 4-repeat tauopathy, including globose-type neurofibrillary tangles, tufted astrocytes, and oligodendroglial coiled bodies as well as TAR DNA-binding protein 43 kDa pathology in association with upper and lower motor neuron degeneration.
|
31319800 |
2019 |
Motor neuron atrophy
|
0.100 |
Biomarker
|
disease |
BEFREE |
Our data indicate TDP43 mutation-dependent but cytosolic aggregation-independent mechanisms of motor neuron degeneration in TDP43 ALS.
|
29630989 |
2018 |
Motor neuron atrophy
|
0.100 |
Biomarker
|
disease |
BEFREE |
However, when microglial cells were depleted, injury-induced motor neuron degeneration follows a characteristic process that includes TDP-43 redistribution into the cytoplasm, axon and extracellular space.
|
29943193 |
2018 |
Motor neuron atrophy
|
0.100 |
Biomarker
|
disease |
BEFREE |
The D2 Dopamine Receptor Interferes With the Protective Effect of the A<sub>2A</sub> Adenosine Receptor on TDP-43 Mislocalization in Experimental Models of Motor Neuron Degeneration.
|
29615863 |
2018 |
Motor neuron atrophy
|
0.100 |
Biomarker
|
disease |
BEFREE |
GANP has been described to function as an mRNA export factor, and to suppress TDP-43-mediated motor neuron degeneration in flies.
|
28633435 |
2017 |
Motor neuron atrophy
|
0.100 |
Biomarker
|
disease |
BEFREE |
Cytoplasmic accumulation of the nuclear protein transactive response DNA-binding protein 43 (TDP-43) is an early determinant of motor neuron degeneration in most amyotrophic lateral sclerosis (ALS) cases.
|
27178390 |
2017 |
Motor neuron atrophy
|
0.100 |
Biomarker
|
disease |
BEFREE |
Interestingly, we report for the first time pathological hallmarks of SPG11 in neurons that include intracytoplasmic granular lysosome-like structures mainly in supratentorial areas, and others in subtentorial areas that are partially reminiscent of those observed in amyotrophic lateral sclerosis, such as ubiquitin and p62 aggregates, except that they are never labelled with anti-TDP-43 or anti-cystatin C. The neuropathological overlap with amyotrophic lateral sclerosis, associated with some shared clinical manifestations, opens up new fields of investigation in the physiopathological continuum of motor neuron degeneration.
|
27016404 |
2016 |
Motor neuron atrophy
|
0.100 |
Biomarker
|
disease |
BEFREE |
Defects in the RNA-binding proteins survival motor neuron (SMN) and TAR DNA-binding protein 43 (TDP-43) cause progressive motor neuron degeneration in spinal muscular atrophy (SMA) and amyotrophic lateral sclerosis (ALS), respectively.
|
27466204 |
2016 |
Motor neuron atrophy
|
0.100 |
Biomarker
|
disease |
BEFREE |
We characterised the abundance of DPRs and their cellular location and compared this to cytoplasmic TDP-43 inclusions in order to explore the role of each inclusion in lower motor neuron degeneration.
|
26108573 |
2015 |
Motor neuron atrophy
|
0.100 |
Biomarker
|
disease |
BEFREE |
TDP-43 is a key disease protein for amyotrophic lateral sclerosis but how it drives motor neuron degeneration remains unresolved.
|
26294190 |
2015 |
Motor neuron atrophy
|
0.100 |
Biomarker
|
disease |
BEFREE |
Age-Dependent TDP-43-Mediated Motor Neuron Degeneration Requires GSK3, hat-trick, and xmas-2.
|
26234214 |
2015 |
Motor neuron atrophy
|
0.100 |
Biomarker
|
disease |
BEFREE |
However, the role of microglia in TDP-43-mediated motor neuron degeneration remains poorly understood.
|
25811799 |
2015 |
Motor neuron atrophy
|
0.100 |
Biomarker
|
disease |
BEFREE |
Loss of TDP-43 causes age-dependent progressive motor neuron degeneration.
|
23449777 |
2013 |
Motor neuron atrophy
|
0.100 |
GeneticVariation
|
disease |
BEFREE |
The molecular mechanisms through which mutant TDP-43 and FUS may cause motor neuron degeneration are not well understood.
|
23559573 |
2013 |
Motor neuron atrophy
|
0.100 |
GeneticVariation
|
disease |
BEFREE |
Our finding suggests that mutant TDP-43 in motor neurons is sufficient to promote the onset and progression of ALS and that motor neuron degeneration is partially reversible, at least in mutant TDP-43 transgenic rats.
|
22156203 |
2012 |
Motor neuron atrophy
|
0.100 |
Biomarker
|
disease |
BEFREE |
These findings indicate that formation of TFG-containing cytoplasmic inclusions and concomitant mislocalization of TDP-43 underlie motor neuron degeneration in HMSN-P. Pathological overlap of proteinopathies involving TFG and TDP-43 highlights a new pathway leading to motor neuron degeneration.
|
22883144 |
2012 |
Motor neuron atrophy
|
0.100 |
GeneticVariation
|
disease |
BEFREE |
We have generated Caenorhabditis elegans and zebrafish animal models expressing mutant human TDP-43 (A315T or G348C) or FUS (S57Δ or R521H) that reflect certain aspects of ALS including motor neuron degeneration, axonal deficits, and progressive paralysis.
|
22848727 |
2012 |
Motor neuron atrophy
|
0.100 |
GeneticVariation
|
disease |
BEFREE |
OPTN-positive inclusions co-localising with TDP-43 were described in SALS and in FALS with SOD-1 mutations, potentially linking two pathologically distinct pathways of motor neuron degeneration.
|
21360076 |
2011 |
Motor neuron atrophy
|
0.100 |
GeneticVariation
|
disease |
BEFREE |
These studies suggest motor neuron degeneration in the mutant SOD1 transgenic mice is associated with TDP-43 histopathology.
|
19379791 |
2009 |
Motor neuron atrophy
|
0.100 |
Biomarker
|
disease |
BEFREE |
FUS is involved in the regulation of transcription and RNA splicing and transport, and it has functional homology to another ALS gene, TARDBP, which suggests that a common mechanism may underlie motor neuron degeneration.
|
19251628 |
2009 |
Motor neuron atrophy
|
0.100 |
Biomarker
|
disease |
BEFREE |
Our findings indicate that the process of motor neuron degeneration in mutant SOD1 transgenic mice is unlikely to involve the abnormalities of TDP-43 described in the human disease.
|
17543992 |
2007 |
Motor neuron atrophy
|
0.100 |
GeneticVariation
|
disease |
BEFREE |
In contrast, the absence of pathological TDP-43 in cases with SOD1 mutations implies that motor neuron degeneration in these cases may result from a different mechanism, and that cases with SOD1 mutations may not be the familial counterpart of sporadic ALS.
|
17469116 |
2007 |