Does a loss of TDP-43 function cause neurodegeneration?
ZS Xu - Molecular neurodegeneration, 2012 - Springer
… Mutations in TDP-43 cause motor neuron degeneration and ALS [28, 29]. The overwhelming
majority of the mutations are located in the C-terminal glycine-rich domain [12], which is …
majority of the mutations are located in the C-terminal glycine-rich domain [12], which is …
Loss of TDP-43 causes age-dependent progressive motor neuron degeneration
Y Iguchi, M Katsuno, J Niwa, S Takagi, S Ishigaki… - Brain, 2013 - academic.oup.com
… of TDP-43 depletion in postnatal mammalian neurons have not … generated motor
neuron-specific TDP-43 knockout (TDP CKO… effects of TDP-43 loss on postnatal motor neurons in …
neuron-specific TDP-43 knockout (TDP CKO… effects of TDP-43 loss on postnatal motor neurons in …
Pathomechanisms of TDP‐43 in neurodegeneration
… similar TDP-43 pathological manifestations in neurons and … , ubiquitinated or
hyperphosphorylated TDP-43 inclusions in … degeneration of motor neurons remains debatable. …
hyperphosphorylated TDP-43 inclusions in … degeneration of motor neurons remains debatable. …
The molecular links between TDP‐43 dysfunction and neurodegeneration
E Buratti, FE Baralle - Advances in genetics, 2009 - Elsevier
… TDP-43 nuclear protein is involved in several major neurodegenerative diseases that include
frontotemporal lobar degeneration … In the neuronal tissues of affected individuals TDP-43 …
frontotemporal lobar degeneration … In the neuronal tissues of affected individuals TDP-43 …
Neuron loss and degeneration in the progression of TDP-43 in frontotemporal lobar degeneration
… and glial intracytoplasmic inclusions as well as accompanying neuron loss. We explored if
cortical neurons detected by NeuN decreased with increasing TDP-43 inclusion pathology in …
cortical neurons detected by NeuN decreased with increasing TDP-43 inclusion pathology in …
[HTML][HTML] Dysregulation of the ALS-associated gene TDP-43 leads to neuronal death and degeneration in mice
… in cortical neurons. Our data … TDP-43 results in loss of normal TDP-43 function(s) and gene
regulatory pathways, culminating in degeneration of selectively vulnerable affected neurons. …
regulatory pathways, culminating in degeneration of selectively vulnerable affected neurons. …
TDP-43-mediated neurodegeneration: towards a loss-of-function hypothesis?
LV Broeck, P Callaerts, B Dermaut - Trends in molecular medicine, 2014 - cell.com
… Loss of normal function of TDP-43 leads to neuronal degeneration. … in the TDP-43 gene
cause ALS. A major unresolved question is whether TDP-43-mediated neuronal loss is caused …
cause ALS. A major unresolved question is whether TDP-43-mediated neuronal loss is caused …
TDP-43: an emerging new player in neurodegenerative diseases
IF Wang, LS Wu, CKJ Shen - Trends in molecular medicine, 2008 - cell.com
… -negative inclusions in frontotemporal lobar degeneration and amyotrophic lateral sclerosis.
… We also propose a model in which loss-of-function of TDP-43 in neurons might contribute to …
… We also propose a model in which loss-of-function of TDP-43 in neurons might contribute to …
Pathological mechanisms underlying TDP-43 driven neurodegeneration in FTLD–ALS spectrum disorders
J Janssens, C Van Broeckhoven - Human molecular genetics, 2013 - academic.oup.com
… Aggregation of misfolded TAR DNA-binding protein 43 (TDP-43) is a striking hallmark of …
lobar degeneration (FTLD) or amyotrophic lateral sclerosis (ALS). A direct causal link with …
lobar degeneration (FTLD) or amyotrophic lateral sclerosis (ALS). A direct causal link with …
Amyotrophic lateral sclerosis and frontotemporal lobar degeneration: a spectrum of TDP‐43 proteinopathies
F Geser, VMY Lee, JQ Trojanowski - Neuropathology, 2010 - Wiley Online Library
… , accumulation and ubiquitination of TDP-43 in the cytoplasm of human motor neurons are
early dysfunctions in the cascade of the events leading to motor neuron degeneration in ALS, …
early dysfunctions in the cascade of the events leading to motor neuron degeneration in ALS, …
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