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Decoding ALS: from genes to mechanism
Amyotrophic lateral sclerosis (ALS) is a progressive and uniformly fatal neurodegenerative
disease. A plethora of genetic factors have been identified that drive the degeneration of …
disease. A plethora of genetic factors have been identified that drive the degeneration of …
Development of multifunctional molecules as potential therapeutic candidates for Alzheimer's disease, Parkinson's disease, and amyotrophic lateral sclerosis in the …
Neurodegenerative diseases pose a substantial socioeconomic burden on society.
Unfortunately, the aging world population and lack of effective cures foreshadow a negative …
Unfortunately, the aging world population and lack of effective cures foreshadow a negative …
Converging mechanisms in ALS and FTD: disrupted RNA and protein homeostasis
Breakthrough discoveries identifying common genetic causes for amyotrophic lateral
sclerosis (ALS) and frontotemporal dementia (FTD) have transformed our view of these …
sclerosis (ALS) and frontotemporal dementia (FTD) have transformed our view of these …
Spreading of pathology in neurodegenerative diseases: a focus on human studies
J Brettschneider, KD Tredici, VMY Lee… - Nature Reviews …, 2015 - nature.com
The progression of many neurodegenerative diseases is thought to be driven by the
template-directed misfolding, seeded aggregation and cell–cell transmission of …
template-directed misfolding, seeded aggregation and cell–cell transmission of …
TDP-43 induces mitochondrial damage and activates the mitochondrial unfolded protein response
Mutations in or dys-regulation of the TDP-43 gene have been associated with TDP-43
proteinopathy, a spectrum of neurodegenerative diseases including Frontotemporal Lobar …
proteinopathy, a spectrum of neurodegenerative diseases including Frontotemporal Lobar …
[HTML][HTML] VDAC1 at the intersection of cell metabolism, apoptosis, and diseases
V Shoshan-Barmatz, A Shteinfer-Kuzmine, A Verma - Biomolecules, 2020 - mdpi.com
The voltage-dependent anion channel 1 (VDAC1) protein, is an important regulator of
mitochondrial function, and serves as a mitochondrial gatekeeper, with responsibility for …
mitochondrial function, and serves as a mitochondrial gatekeeper, with responsibility for …
Mutations in UBQLN2 cause dominant X-linked juvenile and adult-onset ALS and ALS/dementia
Amyotrophic lateral sclerosis (ALS) is a paralytic and usually fatal disorder caused by motor-
neuron degeneration in the brain and spinal cord. Most cases of ALS are sporadic but about …
neuron degeneration in the brain and spinal cord. Most cases of ALS are sporadic but about …
Superoxide dismutase 1 in health and disease: how a frontline antioxidant becomes neurotoxic
Cu/Zn superoxide dismutase (SOD1) is a frontline antioxidant enzyme catalysing superoxide
breakdown and is important for most forms of eukaryotic life. The evolution of aerobic …
breakdown and is important for most forms of eukaryotic life. The evolution of aerobic …
Pathomechanisms of TDP‐43 in neurodegeneration
Neurodegeneration, a term that refers to the progressive loss of structure and function of
neurons, is a feature of many neurodegenerative diseases such as amyotrophic lateral …
neurons, is a feature of many neurodegenerative diseases such as amyotrophic lateral …
Rodent models of amyotrophic lateral sclerosis
Abstract Amyotrophic Lateral Sclerosis (ALS) is a motor neuron disease affecting upper and
lower motor neurons in the central nervous system. Patients with ALS develop extensive …
lower motor neurons in the central nervous system. Patients with ALS develop extensive …