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Molecular mechanisms of TDP-43 misfolding and pathology in amyotrophic lateral sclerosis
TAR DNA binding protein 43 (TDP-43) is a versatile RNA/DNA binding protein involved in
RNA-related metabolism. Hyper-phosphorylated and ubiquitinated TDP-43 deposits act as …
RNA-related metabolism. Hyper-phosphorylated and ubiquitinated TDP-43 deposits act as …
[HTML][HTML] The molecular language of membraneless organelles
Eukaryotic cells organize their intracellular components into organelles that can be
membrane-bound or membraneless. A large number of membraneless organelles, including …
membrane-bound or membraneless. A large number of membraneless organelles, including …
[HTML][HTML] A designer AAV variant permits efficient retrograde access to projection neurons
Efficient retrograde access to projection neurons for the delivery of sensors and effectors
constitutes an important and enabling capability for neural circuit dissection. Such an …
constitutes an important and enabling capability for neural circuit dissection. Such an …
Nuclear-import receptors reverse aberrant phase transitions of RNA-binding proteins with prion-like domains
RNA-binding proteins (RBPs) with prion-like domains (PrLDs) phase transition to functional
liquids, which can mature into aberrant hydrogels composed of pathological fibrils that …
liquids, which can mature into aberrant hydrogels composed of pathological fibrils that …
Protein phase separation: a new phase in cell biology
Cellular compartments and organelles organize biological matter. Most well-known
organelles are separated by a membrane boundary from their surrounding milieu. There are …
organelles are separated by a membrane boundary from their surrounding milieu. There are …
FUS and TDP-43 phases in health and disease
The distinct prion-like domains (PrLDs) of FUS and TDP-43, modulate phase transitions that
result in condensates with a range of material states. These assemblies are implicated in …
result in condensates with a range of material states. These assemblies are implicated in …
Protein misfolding in neurodegenerative diseases: implications and strategies
P Sweeney, H Park, M Baumann, J Dunlop… - Translational …, 2017 - Springer
A hallmark of neurodegenerative proteinopathies is the formation of misfolded protein
aggregates that cause cellular toxicity and contribute to cellular proteostatic collapse …
aggregates that cause cellular toxicity and contribute to cellular proteostatic collapse …
Astrocytes: a central element in neurological diseases
The neurone-centred view of the past disregarded or downplayed the role of astroglia as a
primary component in the pathogenesis of neurological diseases. As this concept is …
primary component in the pathogenesis of neurological diseases. As this concept is …
Liquid-liquid phase separation of TDP-43 and FUS in physiology and pathology of neurodegenerative diseases
JL Carey, L Guo - Frontiers in molecular biosciences, 2022 - frontiersin.org
Liquid-liquid phase separation of RNA-binding proteins mediates the formation of numerous
membraneless organelles with essential cellular function. However, aberrant phase …
membraneless organelles with essential cellular function. However, aberrant phase …
RNA-binding proteins with prion-like domains in health and disease
AF Harrison, J Shorter - Biochemical Journal, 2017 - portlandpress.com
Approximately 70 human RNA-binding proteins (RBPs) contain a prion-like domain (PrLD).
PrLDs are low-complexity domains that possess a similar amino acid composition to prion …
PrLDs are low-complexity domains that possess a similar amino acid composition to prion …