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Neuromuscular junction dysfunction in amyotrophic lateral sclerosis
Amyotrophic lateral sclerosis (ALS) is a fatal neurological disorder characterized by
progressive degeneration of motor neurons leading to skeletal muscle denervation. Earlier …
progressive degeneration of motor neurons leading to skeletal muscle denervation. Earlier …
Specialized subpopulations of deep-layer pyramidal neurons in the neocortex: bridging cellular properties to functional consequences
Neocortical pyramidal neurons with somata in layers 5 and 6 are among the most visually
striking and enigmatic neurons in the brain. These deep-layer pyramidal neurons (DLPNs) …
striking and enigmatic neurons in the brain. These deep-layer pyramidal neurons (DLPNs) …
Motor learning promotes remyelination via new and surviving oligodendrocytes
Oligodendrocyte loss in neurological disease leaves axons vulnerable to damage and
degeneration, and activity-dependent myelination may represent an endogenous …
degeneration, and activity-dependent myelination may represent an endogenous …
Regulation of cortical hyperexcitability in amyotrophic lateral sclerosis: focusing on glial mechanisms
Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disorder
characterized by the loss of both upper and lower motor neurons, resulting in muscle …
characterized by the loss of both upper and lower motor neurons, resulting in muscle …
How do we get from hyperexcitability to excitotoxicity in amyotrophic lateral sclerosis?
Amyotrophic lateral sclerosis is a devastating neurodegenerative disease that, at present,
has no effective cure. Evidence of increased circulating glutamate and hyperexcitability of …
has no effective cure. Evidence of increased circulating glutamate and hyperexcitability of …
Revisiting glutamate excitotoxicity in amyotrophic lateral sclerosis and age-related neurodegeneration
Amyotrophic lateral sclerosis (ALS) is the most common motor neuron disorder. While there
are five FDA-approved drugs for treating this disease, each has only modest benefits. To …
are five FDA-approved drugs for treating this disease, each has only modest benefits. To …
Synaptic dysfunction and plasticity in amyotrophic lateral sclerosis
R Gulino - International Journal of Molecular Sciences, 2023 - mdpi.com
Recent evidence has supported the hypothesis that amyotrophic lateral sclerosis (ALS) is a
multi-step disease, as the onset of symptoms occurs after sequential exposure to a defined …
multi-step disease, as the onset of symptoms occurs after sequential exposure to a defined …
[HTML][HTML] Aberrant axon initial segment plasticity and intrinsic excitability of ALS hiPSC motor neurons
Dysregulated neuronal excitability is a hallmark of amyotrophic lateral sclerosis (ALS). We
sought to investigate how functional changes to the axon initial segment (AIS), the site of …
sought to investigate how functional changes to the axon initial segment (AIS), the site of …
Cytoplasmic FUS triggers early behavioral alterations linked to cortical neuronal hyperactivity and inhibitory synaptic defects
J Scekic-Zahirovic, I Sanjuan-Ruiz, V Kan… - Nature …, 2021 - nature.com
Gene mutations causing cytoplasmic mislocalization of the RNA-binding protein FUS lead to
severe forms of amyotrophic lateral sclerosis (ALS). Cytoplasmic accumulation of FUS is …
severe forms of amyotrophic lateral sclerosis (ALS). Cytoplasmic accumulation of FUS is …
[HTML][HTML] Modeling cell-autonomous motor neuron phenotypes in ALS using iPSCs
J Hawrot, S Imhof, BJ Wainger - Neurobiology of disease, 2020 - Elsevier
Amyotrophic lateral sclerosis (ALS) is an aggressive and uniformly fatal degenerative
disease of the motor nervous system. In order to understand underlying disease …
disease of the motor nervous system. In order to understand underlying disease …