[HTML][HTML] Homozygous ALS-linked mutations in TARDBP/TDP-43 lead to hypoactivity and synaptic abnormalities in human iPSC-derived motor neurons
S Lépine, A Nauleau-Javaudin, E Deneault, CXQ Chen… - Iscience, 2024 - cell.com
Cytoplasmic mislocalization and aggregation of the RNA-binding protein TDP-43 is a
pathological hallmark of the motor neuron (MN) disease amyotrophic lateral sclerosis (ALS) …
pathological hallmark of the motor neuron (MN) disease amyotrophic lateral sclerosis (ALS) …
Homozygous ALS-linked mutations in TARDBP/TDP-43 lead to progressive synaptic dysfunction in human iPSC-derived motor neurons
S Lépine, A Nauleau-Javaudin, E Deneault, CXQ Chen… - bioRxiv, 2023 - biorxiv.org
Cytoplasmic mislocalization and aggregation of the RNA-binding protein TDP-43 is a
pathological hallmark of the motor neuron (MN) disease amyotrophic lateral sclerosis (ALS) …
pathological hallmark of the motor neuron (MN) disease amyotrophic lateral sclerosis (ALS) …
Mutant TDP-43 causes early-stage dose-dependent motor neuron degeneration in a TARDBP knockin mouse model of ALS
SY Ebstein, I Yagudayeva, NA Shneider - Cell reports, 2019 - cell.com
Rare mutations in TARDBP, the gene encoding TDP-43, cause amyotrophic lateral sclerosis
(ALS), and TDP-43 pathology is seen in a large majority of ALS patients, suggesting a …
(ALS), and TDP-43 pathology is seen in a large majority of ALS patients, suggesting a …
TDP-43 gains function due to perturbed autoregulation in a Tardbp knock-in mouse model of ALS-FTD
Amyotrophic lateral sclerosis–frontotemporal dementia (ALS-FTD) constitutes a devastating
disease spectrum characterized by 43-kDa TAR DNA-binding protein (TDP-43) pathology …
disease spectrum characterized by 43-kDa TAR DNA-binding protein (TDP-43) pathology …
[HTML][HTML] The vulnerability of motor and frontal cortex-dependent behaviors in mice expressing ALS-linked mutation in TDP-43
TDP-43 aggregates are the defining pathological hallmark for amyotrophic lateral sclerosis
(ALS) and frontotemporal dementia (FTD). Strikingly, these TDP-43 proteinopathies are also …
(ALS) and frontotemporal dementia (FTD). Strikingly, these TDP-43 proteinopathies are also …
Wild type human TDP-43 potentiates ALS-linked mutant TDP-43 driven progressive motor and cortical neuron degeneration with pathological features of ALS
JC Mitchell, R Constable, E So, C Vance… - Acta neuropathologica …, 2015 - Springer
Introduction Amyotrophic lateral sclerosis (ALS) is a relentlessly progressive
neurodegenerative disorder, and cytoplasmic inclusions containing transactive response …
neurodegenerative disorder, and cytoplasmic inclusions containing transactive response …
Cytoplasmic human TDP-43 mislocalization induces widespread dendritic spine loss in mouse upper motor neurons
MS Dyer, A Woodhouse, CA Blizzard - Brain Sciences, 2021 - mdpi.com
Amyotrophic lateral sclerosis (ALS) is defined by the destruction of upper-and lower motor
neurons. Post-mortem, nearly all ALS cases are positive for cytoplasmic aggregates …
neurons. Post-mortem, nearly all ALS cases are positive for cytoplasmic aggregates …
A robust TDP-43 knock-in mouse model of ALS
Amyotrophic lateral sclerosis (ALS) is a fatal, adult-onset degenerative disorder of motor
neurons. The diseased spinal cord motor neurons of more than 95% of amyotrophic lateral …
neurons. The diseased spinal cord motor neurons of more than 95% of amyotrophic lateral …
[HTML][HTML] Selective spatiotemporal vulnerability of central nervous system neurons to pathologic TAR DNA-binding protein 43 in aged transgenic mice
Amyotrophic lateral sclerosis (ALS) is a rapidly progressing and fatal disease characterized
by muscular atrophy because of loss of upper and lower motor neurons. Histopathologically …
by muscular atrophy because of loss of upper and lower motor neurons. Histopathologically …
Mutations in TDP-43 link glycine-rich domain functions to amyotrophic lateral sclerosis
GS Pesiridis, VMY Lee… - Human molecular …, 2009 - academic.oup.com
Amyotrophic lateral sclerosis (ALS) is the most common adult motor neuron disease that
affects∼ 2/100 000 individuals each year worldwide. Patients with ALS suffer from rapidly …
affects∼ 2/100 000 individuals each year worldwide. Patients with ALS suffer from rapidly …
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