The neurobiology of amyotrophic lateral sclerosis

A Bento‐Abreu, P Van Damme… - European Journal of …, 2010 - Wiley Online Library
Amyotrophic lateral sclerosis is a degenerative disease affecting the motor neurons. In spite
of our growing insights into its biology, it remains a lethal condition. The identification of the …

Potential Effect of S-Nitrosylated Protein Disulfide Isomerase on Mutant SOD1 Aggregation and Neuronal Cell Death in Amyotrophic Lateral Sclerosis

GS Jeon, T Nakamura, JS Lee, WJ Choi, SW Ahn… - Molecular …, 2014 - Springer
Aggregation of misfolded protein and resultant intracellular inclusion body formation are
common hallmarks of mutant superoxide dismutase (mSOD1)-linked familial amyotrophic …

Activation of the Wnt/β-catenin signaling pathway is associated with glial proliferation in the adult spinal cord of ALS transgenic mice

Y Chen, Y Guan, H Liu, X Wu, L Yu, S Wang… - Biochemical and …, 2012 - Elsevier
Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease characterized by the
progressive and fatal loss of motor neurons. In ALS, there is a significant cell proliferation in …

[HTML][HTML] PP2A and GSK3 act as modifiers of FUS-ALS by modulating mitochondrial transport

P Tziortzouda, J Steyaert, W Scheveneels, A Sicart… - Acta …, 2024 - Springer
Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disease which currently
lacks effective treatments. Mutations in the RNA-binding protein FUS are a common cause of …

[HTML][HTML] Decreased mitochondrial function, biogenesis, and degradation in peripheral blood mononuclear cells from amyotrophic lateral sclerosis patients as a …

BG Araujo, LF Souza e Silva, JL de Barros Torresi… - Molecular …, 2020 - Springer
Amyotrophic lateral sclerosis (ALS) is a multifactorial and progressive neurodegenerative
disease of unknown etiology. Due to ALS's unpredictable onset and progression rate, the …

[HTML][HTML] Muscleblind acts as a modifier of FUS toxicity by modulating stress granule dynamics and SMN localization

I Casci, K Krishnamurthy, S Kour, V Tripathy… - Nature …, 2019 - nature.com
Mutations in fused in sarcoma (FUS) lead to amyotrophic lateral sclerosis (ALS) with varying
ages of onset, progression and severity. This suggests that unknown genetic factors …

Glycoursodeoxycholic acid reduces matrix metalloproteinase-9 and caspase-9 activation in a cellular model of superoxide dismutase-1 neurodegeneration

AR Vaz, C Cunha, C Gomes, N Schmucki… - Molecular …, 2015 - Springer
Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disease that affects mainly
motor neurons (MNs). NSC-34 MN-like cells carrying the G93A mutation in human …

Wnt signaling is altered by spinal cord neuronal dysfunction in amyotrophic lateral sclerosis transgenic mice

L Yu, Y Guan, X Wu, Y Chen, Z Liu, H Du… - Neurochemical research, 2013 - Springer
Amyotrophic lateral sclerosis (ALS) is a chronic neurodegenerative disease characterized
by progressive degeneration of the motor neurons in the cortex, brainstem, and spinal cord …

Human motor neuron generation from embryonic stem cells and induced pluripotent stem cells

M Nizzardo, C Simone, M Falcone, F Locatelli… - Cellular and Molecular …, 2010 - Springer
Motor neuron diseases (MNDs) are a group of neurological disorders that selectively affect
motor neurons. There are currently no cures or efficacious treatments for these diseases. In …

[HTML][HTML] MicroRNA implications across neurodevelopment and neuropathology

S Martino, I Di Girolamo, A Orlacchio… - BioMed Research …, 2009 - Wiley Online Library
MicroRNAs (miRNAs) have rapidly emerged as biologically important mediators of
posttranscriptional and epigenetic regulation in both plants and animals. miRNAs function …