Reduced oxidative damage in ALS by high‐dose enteral melatonin treatment

JH Weishaupt, C Bartels, E Pölking… - Journal of pineal …, 2006 - Wiley Online Library
Amyotrophic lateral sclerosis (ALS) is the collective term for a fatal motoneuron disease of
different etiologies, with oxidative stress as a common molecular denominator of disease …

Nuclear transport impairment of amyotrophic lateral sclerosis‐linked mutations in FUS/TLS

D Ito, M Seki, Y Tsunoda, H Uchiyama… - Annals of …, 2011 - Wiley Online Library
Objective: The fused in sarcoma/translated in liposarcoma (FUS/TLS) protein was recently
identified as a cause of familial amyotrophic lateral sclerosis (ALS), as well as a major …

Mutant SOD1 alters the motor neuronal transcriptome: implications for familial ALS

J Kirby, E Halligan, MJ Baptista, S Allen, PR Heath… - Brain, 2005 - academic.oup.com
Familial amyotrophic lateral sclerosis (FALS) is caused, in 20% of cases, by mutations in the
Cu/Zn superoxide dismutase gene (SOD1). Although motor neuron injury occurs through a …

Mutant SOD1 in neuronal mitochondria causes toxicity and mitochondrial dynamics abnormalities

J Magrané, I Hervias, MS Henning… - Human molecular …, 2009 - academic.oup.com
Amyotrophic lateral sclerosis (ALS) is a fatal neurological disorder characterized by motor
neuron degeneration. Mutations in Cu, Zn-superoxide dismutase (SOD1) are responsible for …

The overexpression of TDP-43 in astrocytes causes neurodegeneration via a PTP1B-mediated inflammatory response

S Lee, S Kim, HY Kang, HR Lim, Y Kwon, M Jo… - Journal of …, 2020 - Springer
Background Cytoplasmic inclusions of transactive response DNA binding protein of 43 kDa
(TDP-43) in neurons and astrocytes are a feature of some neurodegenerative diseases …

Progranulin is expressed within motor neurons and promotes neuronal cell survival

CL Ryan, DC Baranowski, BP Chitramuthu, S Malik… - BMC neuroscience, 2009 - Springer
Background Progranulin is a secreted high molecular weight growth factor bearing seven
and one half copies of the cysteine-rich granulin-epithelin motif. While inappropriate over …

Mitochondrial defects in the respiratory complex I contribute to impaired translational initiation via ROS and energy homeostasis in SMA motor neurons

MP Thelen, B Wirth, MJ Kye - Acta neuropathologica communications, 2020 - Springer
Spinal muscular atrophy (SMA) is a neuromuscular disease characterized by loss of lower
motor neurons, which leads to proximal muscle weakness and atrophy. SMA is caused by …

Differentiated NSC-34 motoneuron-like cells as experimental model for cholinergic neurodegeneration

O Maier, J Böhm, M Dahm, S Brück, C Beyer… - Neurochemistry …, 2013 - Elsevier
Alpha-motoneurons appear to be exceedingly affected in neurodegenerative diseases such
as amyotrophic lateral sclerosis (ALS). Morphological and physiological degeneration of this …

Thermosensitive chitosan-based hydrogels supporting motor neuron-like NSC-34 cell differentiation

A Stanzione, A Polini, V La Pesa, A Quattrini… - Biomaterials …, 2021 - pubs.rsc.org
Motor neuron diseases are neurodegenerative diseases that predominantly affect the
neuromuscular system. To date, there are no valid therapeutic treatments for such diseases …

Sigma-1 receptor chaperones rescue nucleocytoplasmic transport deficit seen in cellular and Drosophila ALS/FTD models

PT Lee, JC Liévens, SM Wang, JY Chuang… - Nature …, 2020 - nature.com
In a subgroup of patients with amyotrophic lateral sclerosis (ALS)/Frontotemporal dementia
(FTD), the (G4C2)-RNA repeat expansion from C9orf72 chromosome binds to the Ran …