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The role of TDP-43 mislocalization in amyotrophic lateral sclerosis
Since its discovery as a primary component in cytoplasmic aggregates in post-mortem tissue
of patients with Amyotrophic Lateral Sclerosis (ALS), TAR DNA Binding Protein 43 kDa (TDP …
of patients with Amyotrophic Lateral Sclerosis (ALS), TAR DNA Binding Protein 43 kDa (TDP …
ALS genetics: gains, losses, and implications for future therapies
Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disorder caused by the loss
of motor neurons from the brain and spinal cord. The ALS community has made remarkable …
of motor neurons from the brain and spinal cord. The ALS community has made remarkable …
An update on genetic frontotemporal dementia
Frontotemporal dementia (FTD) is a highly heritable group of neurodegenerative disorders,
with around 30% of patients having a strong family history. The majority of that heritability is …
with around 30% of patients having a strong family history. The majority of that heritability is …
Immune dysregulation in amyotrophic lateral sclerosis: mechanisms and emerging therapies
DR Beers, SH Appel - The Lancet Neurology, 2019 - thelancet.com
Neuroinflammation is a common pathological feature of many neurodegenerative diseases,
including amyotrophic lateral sclerosis (ALS), and is characterised by activated CNS …
including amyotrophic lateral sclerosis (ALS), and is characterised by activated CNS …
The overlap** genetics of amyotrophic lateral sclerosis and frontotemporal dementia
Amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD) are two diseases
that form a broad neurodegenerative continuum. Considerable effort has been made to …
that form a broad neurodegenerative continuum. Considerable effort has been made to …
Recent updates on the genetics of amyotrophic lateral sclerosis and frontotemporal dementia
Amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD) primarily affect the
motor and frontotemporal areas of the brain, respectively. These disorders share clinical …
motor and frontotemporal areas of the brain, respectively. These disorders share clinical …
Development of disease-modifying drugs for frontotemporal dementia spectrum disorders
Frontotemporal dementia (FTD) encompasses a spectrum of clinical syndromes
characterized by progressive executive, behavioural and language dysfunction. The various …
characterized by progressive executive, behavioural and language dysfunction. The various …
ALS-and FTD-associated missense mutations in TBK1 differentially disrupt mitophagy
TANK-binding kinase 1 (TBK1) is a multifunctional kinase with an essential role in
mitophagy, the selective clearance of damaged mitochondria. More than 90 distinct …
mitophagy, the selective clearance of damaged mitochondria. More than 90 distinct …
Autophagic and endo-lysosomal dysfunction in neurodegenerative disease
Due to their post-mitotic state, metabolic demands and often large polarised morphology, the
function and survival of neurons is dependent on an efficient cellular waste clearance …
function and survival of neurons is dependent on an efficient cellular waste clearance …
The loss of TBK1 kinase activity in motor neurons or in all cell types differentially impacts ALS disease progression in SOD1 mice
DNA sequence variants in the TBK1 gene associate with or cause sporadic or familial
amyotrophic lateral sclerosis (ALS). Here we show that mice bearing human ALS-associated …
amyotrophic lateral sclerosis (ALS). Here we show that mice bearing human ALS-associated …