ALS genetics, mechanisms, and therapeutics: where are we now?
The scientific landscape surrounding amyotrophic lateral sclerosis (ALS) continues to shift
as the number of genes associated with the disease risk and pathogenesis, and the cellular …
as the number of genes associated with the disease risk and pathogenesis, and the cellular …
The role of TDP-43 mislocalization in amyotrophic lateral sclerosis
TR Suk, MWC Rousseaux - Molecular neurodegeneration, 2020 - Springer
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 …
PINK1-PRKN mediated mitophagy: differences between in vitro and in vivo models
R Han, Y Liu, S Li, XJ Li, W Yang - Autophagy, 2023 - Taylor & Francis
Mitophagy is a key intracellular process that selectively removes damaged mitochondria to
prevent their accumulation that can cause neuronal degeneration. During mitophagy, PINK1 …
prevent their accumulation that can cause neuronal degeneration. During mitophagy, PINK1 …
The pathobiology of TDP-43 C-terminal fragments in ALS and FTLD
BA Berning, AK Walker - Frontiers in neuroscience, 2019 - frontiersin.org
During neurodegenerative disease, the multifunctional RNA-binding protein TDP-43
undergoes a vast array of post-translational modifications, including phosphorylation …
undergoes a vast array of post-translational modifications, including phosphorylation …
[HTML][HTML] Deficiency of parkin causes neurodegeneration and accumulation of pathological α-synuclein in monkey models
R Han, Q Wang, X Xiong, X Chen, Z Tu, B Li… - The Journal of Clinical …, 2024 - jci.org
Parkinson's disease (PD) is characterized by age-dependent neurodegeneration and the
accumulation of toxic phosphorylated α-synuclein (pS129-α-syn). The mechanisms …
accumulation of toxic phosphorylated α-synuclein (pS129-α-syn). The mechanisms …
Cell‐to‐cell transmission of C9orf72 poly‐(Gly‐Ala) triggers key features of ALS/FTD
B Khosravi, KD LaClair, H Riemenschneider… - The EMBO …, 2020 - embopress.org
The C9orf72 repeat expansion causes amyotrophic lateral sclerosis and frontotemporal
dementia, but the poor correlation between C9orf72‐specific pathology and TDP‐43 …
dementia, but the poor correlation between C9orf72‐specific pathology and TDP‐43 …
Congenic expression of poly-GA but not poly-PR in mice triggers selective neuron loss and interferon responses found in C9orf72 ALS
KD LaClair, Q Zhou, M Michaelsen, B Wefers… - Acta …, 2020 - Springer
Expansion of a (G 4 C 2) n repeat in C9orf72 causes amyotrophic lateral sclerosis (ALS) and
frontotemporal dementia (FTD), but the link of the five repeat-encoded dipeptide repeat …
frontotemporal dementia (FTD), but the link of the five repeat-encoded dipeptide repeat …
Mechanisms and consequences of noncanonical inflammasome-mediated pyroptosis
SS Wright, SO Vasudevan, VA Rathinam - Journal of molecular biology, 2022 - Elsevier
The noncanonical inflammasome, comprising inflammatory caspases 4, 5, or 11, monitors
the cytosol for bacterial lipopolysaccharide (LPS). Intracellular LPS-elicited autoproteolysis …
the cytosol for bacterial lipopolysaccharide (LPS). Intracellular LPS-elicited autoproteolysis …
Pyroptosis by caspase-11 inflammasome-Gasdermin D pathway in autoimmune diseases
J Wu, J Sun, X Meng - Pharmacological Research, 2021 - Elsevier
Inflammasomes are a group of supramolecular complexes primarily comprise a sensor,
adaptor protein and an effector. Among them, canonical inflammasomes are assembled by …
adaptor protein and an effector. Among them, canonical inflammasomes are assembled by …
Genetically modified large animal models for investigating neurodegenerative diseases
Neurodegenerative diseases represent a large group of neurological disorders including
Alzheimer's disease, amyotrophic lateral sclerosis, Parkinson's disease, and Huntington's …
Alzheimer's disease, amyotrophic lateral sclerosis, Parkinson's disease, and Huntington's …