Impaired glymphatic function in the early stages of disease in a TDP-43 mouse model of amyotrophic lateral sclerosis
Background Multiple lines of evidence suggest possible impairment of the glymphatic
system in amyotrophic lateral sclerosis (ALS). To investigate this, we used in vivo magnetic …
system in amyotrophic lateral sclerosis (ALS). To investigate this, we used in vivo magnetic …
Recapitulation of pathological TDP-43 features in immortalized lymphocytes from sporadic ALS patients
D Posa, L Martínez-González, F Bartolomé… - Molecular …, 2019 - Springer
Amyotrophic lateral sclerosis (ALS) is a fatal progressive neurodegenerative disorder of still
unknown etiology that results in loss of motoneurons, paralysis, and death, usually between …
unknown etiology that results in loss of motoneurons, paralysis, and death, usually between …
Extensive phenotypic characterisation of a human TDP-43Q331K transgenic mouse model of amyotrophic lateral sclerosis (ALS)
The majority of preclinical studies in ALS have relied on transgenic models with
overexpression of mutant human superoxide dismutase 1 (SOD1), widely regarded to have …
overexpression of mutant human superoxide dismutase 1 (SOD1), widely regarded to have …
Neuromuscular junction denervation and terminal Schwann cell loss in the hTDP‐43 overexpression mouse model of amyotrophic lateral sclerosis
A Alhindi, M Shand, HL Smith, AS Leite… - Neuropathology and …, 2023 - Wiley Online Library
Aims Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disease with complex
aetiology. Despite evidence of neuromuscular junction (NMJ) denervation and 'dying …
aetiology. Despite evidence of neuromuscular junction (NMJ) denervation and 'dying …
Selective motor neuron resistance and recovery in a new inducible mouse model of TDP-43 proteinopathy
KJ Spiller, CJ Cheung, CR Restrepo… - Journal of …, 2016 - Soc Neuroscience
Motor neurons (MNs) are the neuronal class that is principally affected in amyotrophic lateral
sclerosis (ALS), but it is widely known that individual motor pools do not succumb to …
sclerosis (ALS), but it is widely known that individual motor pools do not succumb to …
The glymphatic system and Amyotrophic lateral sclerosis
The glymphatic system and the meningeal lymphatic vessels provide a pathway for transport
of solutes and clearance of toxic material from the brain. Of specific relevance to ALS, this is …
of solutes and clearance of toxic material from the brain. Of specific relevance to ALS, this is …
Abnormal regenerative responses and impaired axonal outgrowth after nerve crush in TDP-43 transgenic mouse models of amyotrophic lateral sclerosis
Tar DNA binding protein 43 (TDP-43) mislocalization and aggregation is a hallmark of
amyotrophic lateral sclerosis (ALS) and frontotemporal lobar dementia. Moreover, TDP-43 …
amyotrophic lateral sclerosis (ALS) and frontotemporal lobar dementia. Moreover, TDP-43 …
Deregulation of TDP-43 in amyotrophic lateral sclerosis triggers nuclear factor κB–mediated pathogenic pathways
TDP-43 (TAR DNA-binding protein 43) inclusions are a hallmark of amyotrophic lateral
sclerosis (ALS). In this study, we report that TDP-43 and nuclear factor κB (NF-κB) p65 …
sclerosis (ALS). In this study, we report that TDP-43 and nuclear factor κB (NF-κB) p65 …
Mice carrying ALS mutant TDP-43, but not mutant FUS, display in vivo defects in axonal transport of signaling endosomes
Amyotrophic lateral sclerosis (ALS) is a fatal, progressive neurodegenerative disease
resulting from a complex interplay between genetics and environment. Impairments in …
resulting from a complex interplay between genetics and environment. Impairments in …
Microglial CD68 and L-ferritin upregulation in response to phosphorylated-TDP-43 pathology in the amyotrophic lateral sclerosis brain
Microglia, the innate immune cells of the brain, are activated by damage or disease. In
mouse models of amyotrophic lateral sclerosis (ALS), microglia shift from neurotrophic to …
mouse models of amyotrophic lateral sclerosis (ALS), microglia shift from neurotrophic to …