Dysregulation of lncRNAs during skeletal muscle development in a patient-derived in vitro model of amyotrophic lateral sclerosis

2021 ◽  
Vol 429 ◽  
pp. 119399
Author(s):  
Silvia Bonanno ◽  
Eleonora Giagnorio ◽  
Claudia Malacarne ◽  
Patrizia Bossolasco ◽  
Erika Salvi ◽  
...  
Keyword(s):  
Skeletal Muscle ◽  
Amyotrophic Lateral Sclerosis ◽  
In Vitro Model ◽  
Muscle Development ◽  
Skeletal Muscle Development ◽  
Vitro Model ◽  
Lateral Sclerosis

scholarly journals 3-D in vitro model of early skeletal muscle development. Cell Motil Cytoskeleton 54:226-236, 2003

2003 ◽  
Vol 55 (4) ◽  
pp. 278-278
Author(s):  
U. Cheema ◽  
S-Y. Yang ◽  
V. Mudera ◽  
G. Goldspink ◽  
R.A. Brown
Keyword(s):  
Skeletal Muscle ◽  
In Vitro Model ◽  
Muscle Development ◽  
Skeletal Muscle Development ◽  
Vitro Model

3-D in vitro model of early skeletal muscle development

2003 ◽  
Vol 54 (3) ◽  
pp. 226-236 ◽  
Author(s):  
U. Cheema ◽  
S.-Y. Yang ◽  
V. Mudera ◽  
G.G. Goldspink ◽  
R.A. Brown
Keyword(s):  
Skeletal Muscle ◽  
In Vitro Model ◽  
Muscle Development ◽  
Skeletal Muscle Development ◽  
Vitro Model

Molecular mechanisms involved in the protective effect of pituitary adenylate cyclase-activating polypeptide in an in vitro model of amyotrophic lateral sclerosis

2018 ◽  
Vol 234 (4) ◽  
pp. 5203-5214 ◽  
Author(s):  
Grazia Maugeri ◽  
Agata G. D’Amico ◽  
Daniela M. Rasà ◽  
Concetta Federico ◽  
Salvatore Saccone ◽  
...  
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Adenylate Cyclase ◽  
Protective Effect ◽  
In Vitro Model ◽  
Molecular Mechanisms ◽  
Pituitary Adenylate Cyclase ◽  
Vitro Model ◽  
Lateral Sclerosis

Exosome derived from murine adipose-derived stromal cells: Neuroprotective effect on in vitro model of amyotrophic lateral sclerosis

2016 ◽  
Vol 340 (1) ◽  
pp. 150-158 ◽  
Author(s):  
Roberta Bonafede ◽  
Ilaria Scambi ◽  
Daniele Peroni ◽  
Valentina Potrich ◽  
Federico Boschi ◽  
...  
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Stromal Cells ◽  
In Vitro Model ◽  
Neuroprotective Effect ◽  
Adipose Derived Stromal Cells ◽  
Vitro Model ◽  
Lateral Sclerosis

scholarly journals PACAP Modulates the Autophagy Process in an In Vitro Model of Amyotrophic Lateral Sclerosis

2020 ◽  
Vol 21 (8) ◽  
pp. 2943 ◽  
Author(s):  
Agata Grazia D’Amico ◽  
Grazia Maugeri ◽  
Salvatore Saccone ◽  
Concetta Federico ◽  
Sebastiano Cavallaro ◽  
...  
Keyword(s):  
Cell Death ◽  
Amyotrophic Lateral Sclerosis ◽  
Motor Neuron ◽  
Motor Neurons ◽  
In Vitro Model ◽  
Erk Signaling ◽  
Hypoxic Stress ◽  
Vitro Model ◽  
Lateral Sclerosis

Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disease of complex etiology leading to motor neuron degeneration. Many gene alterations cause this pathology, including mutation in Cu, Zn superoxide dismutase (SOD1), which leads to its gain of function. Mutant SOD1 proteins are prone to aberrant misfolding and create aggregates that impair autophagy. The hypoxic stress is strictly linked to the disease progression since it induces uncontrolled autophagy activation and the consequent high rates of cell death. Previously, we showed that pituitary adenylate cyclase-activating polypeptide (PACAP) exerts neurotrophic activity in cultured mSOD1 motor neurons exposed to serum deprivation. To date, no studies have examined whether the protective effect of PACAP on mSOD1 cells exposed to hypoxic insult is mediated through the regulation of the autophagy process. In the present study, we used the neuroblastoma-spinal cord-34 (NSC-34) cell line, stably expressing human wild type or mutant SOD1 G93A, to represent a well characterized in vitro model of a familial form of ALS. These cells were exposed to 100-µM desferrioxamine mesylate salt for 24h, to mimic the hypoxic stress affecting motor neurons during the disease progression. Our results showed that PACAP treatment significantly reduced cell death and hypoxia-induced mSOD1 accumulation by modulating the autophagy process in G93A motor neurons, as revealed by the decreased LC3II and the increased p62 levels, two autophagy indicators. These results were also confirmed by evaluating the vacuole formation detected through light chain 3 (LC3) immunofluorescence. Furthermore, the PACAP effects on autophagy seem to be mediated through the activation of the MAPK/ERK signaling pathway. Overall, our data demonstrated that PACAP exerts an ameliorative effect on the mSOD1 motor neuron viability by modulating a hypoxia-induced autophagy process through activation of MAPK/ERK signaling cascade.

Gingival Stromal Cells as an In Vitro Model: Cannabidiol Modulates Genes Linked With Amyotrophic Lateral Sclerosis

2016 ◽  
Vol 118 (4) ◽  
pp. 819-828 ◽  
Author(s):  
Thangavelu Soundara Rajan ◽  
Domenico Scionti ◽  
Francesca Diomede ◽  
Gianpaolo Grassi ◽  
Federica Pollastro ◽  
...  
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Stromal Cells ◽  
In Vitro Model ◽  
Vitro Model ◽  
Lateral Sclerosis
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