DNA damage as a mechanism of neurodegeneration in ALS and a contributor to astrocyte toxicity
JR Kok, NM Palminha, C Dos Santos Souza… - Cellular and Molecular …, 2021 - Springer
Increasing evidence supports the involvement of DNA damage in several
neurodegenerative diseases, including amyotrophic lateral sclerosis (ALS). Elevated levels …
neurodegenerative diseases, including amyotrophic lateral sclerosis (ALS). Elevated levels …
Extracellular vesicles and amyotrophic lateral sclerosis: from misfolded protein vehicles to promising clinical biomarkers
D Gagliardi, N Bresolin, GP Comi, S Corti - Cellular and Molecular Life …, 2021 - Springer
Extracellular vesicles (EVs) are small reservoirs of different molecules and important
mediators of cell-to-cell communication. As putative vehicles of misfolded protein …
mediators of cell-to-cell communication. As putative vehicles of misfolded protein …
Integrative proteomics highlight presynaptic alterations and c-Jun misactivation as convergent pathomechanisms in ALS
A Aly, ZI Laszlo, S Rajkumar, T Demir, N Hindley… - Acta …, 2023 - Springer
Abstract Amyotrophic Lateral Sclerosis (ALS) is a fatal neurodegenerative disease mainly
affecting upper and lower motoneurons. Several functionally heterogeneous genes have …
affecting upper and lower motoneurons. Several functionally heterogeneous genes have …
Repeat length of C9orf72-associated glycine–alanine polypeptides affects their toxicity
J Morón-Oset, LKS Fischer, N Jauré, P Zhang… - Acta Neuropathologica …, 2023 - Springer
G4C2 hexanucleotide repeat expansions in a non-coding region of the C9orf72 gene are
the most common cause of familial amyotrophic lateral sclerosis (ALS) and frontotemporal …
the most common cause of familial amyotrophic lateral sclerosis (ALS) and frontotemporal …
Stable transgenic C9orf72 zebrafish model key aspects of the ALS/FTD phenotype and reveal novel pathological features
MP Shaw, A Higginbottom, A McGown… - Acta neuropathologica …, 2018 - Springer
A hexanucleotide repeat expansion (HRE) within the chromosome 9 open reading frame 72
(C9orf72) gene is the most prevalent cause of amyotrophic lateral sclerosis/fronto-temporal …
(C9orf72) gene is the most prevalent cause of amyotrophic lateral sclerosis/fronto-temporal …
A systematic and comprehensive review on disease-causing genes in amyotrophic lateral sclerosis
E Srinivasan, R Rajasekaran - Journal of Molecular Neuroscience, 2020 - Springer
Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disorder and is characterized by
degeneration and axon loss from the upper motor neuron, that descends from the lower …
degeneration and axon loss from the upper motor neuron, that descends from the lower …
Glycine-alanine dipeptide repeats spread rapidly in a repeat length-and age-dependent manner in the fly brain
J Morón-Oset, T Supèr, J Esser, AM Isaacs… - Acta neuropathologica …, 2019 - Springer
Hexanucleotide repeat expansions of variable size in C9orf72 are the most prevalent
genetic cause of amyotrophic lateral sclerosis and frontotemporal dementia. Sense and …
genetic cause of amyotrophic lateral sclerosis and frontotemporal dementia. Sense and …
Phenotypic diversity in ALS and the role of poly-conformational protein misfolding
JI Ayers, DR Borchelt - Acta neuropathologica, 2021 - Springer
In many types of familial amyotrophic lateral sclerosis (fALS), mutations cause proteins to
gain toxic properties that mediate neurodegenerative processes. It is becoming increasingly …
gain toxic properties that mediate neurodegenerative processes. It is becoming increasingly …
Prion-like properties of disease-relevant proteins in amyotrophic lateral sclerosis
S Bräuer, V Zimyanin, A Hermann - Journal of Neural Transmission, 2018 - Springer
The hallmark of age-related neurodegenerative diseases is the appearance of cellular
protein deposits and spreading of this pathology throughout the central nervous system …
protein deposits and spreading of this pathology throughout the central nervous system …
The MuSK agonist antibody protects the neuromuscular junction and extends the lifespan in C9orf72-ALS mice
S Sun, Y Shen, X Zhang, N Ding, Z Xu, Q Zhang, L Li - Molecular Therapy, 2024 - cell.com
The disassembly of the neuromuscular junction (NMJ) is an early event in amyotrophic
lateral sclerosis (ALS), ultimately leading to motor dysfunction and lethal respiratory …
lateral sclerosis (ALS), ultimately leading to motor dysfunction and lethal respiratory …