scholarly journals The Overexpression of TDP-43 Protein in the Neuron and Oligodendrocyte Cells Causes the Progressive Motor Neuron Degeneration in the SOD1 G93A Transgenic Mouse Model of Amyotrophic Lateral Sclerosis

2016 ◽  
Vol 12 (9) ◽  
pp. 1140-1149 ◽  
Author(s):  
Yi Lu ◽  
Chunyan Tang ◽  
Lei Zhu ◽  
Jiao Li ◽  
Huiting Liang ◽  
...  
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Mouse Model ◽  
Motor Neuron ◽  
Transgenic Mouse ◽  
Transgenic Mouse Model ◽  
Motor Neuron Degeneration ◽  
Neuron Degeneration ◽  
Lateral Sclerosis

Increased ER stress during motor neuron degeneration in a transgenic mouse model of amyotrophic lateral sclerosis

2007 ◽  
Vol 29 (8) ◽  
pp. 767-771 ◽  
Author(s):  
Tetsuya Nagata ◽  
Hristelina Ilieva ◽  
Tetsuro Murakami ◽  
Mito Shiote ◽  
Hisashi Narai ◽  
...  
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Mouse Model ◽  
Er Stress ◽  
Motor Neuron ◽  
Transgenic Mouse ◽  
Transgenic Mouse Model ◽  
Motor Neuron Degeneration ◽  
Neuron Degeneration ◽  
Lateral Sclerosis

scholarly journals Loss of Sarm1 does not suppress motor neuron degeneration in the SOD1G93A mouse model of amyotrophic lateral sclerosis

2018 ◽  
Vol 27 (21) ◽  
pp. 3761-3771 ◽  
Author(s):  
Owen M Peters ◽  
Elizabeth A Lewis ◽  
Jeannette M Osterloh ◽  
Alexandra Weiss ◽  
Johnny S Salameh ◽  
...  
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Mouse Model ◽  
Motor Neuron ◽  
Motor Neuron Degeneration ◽  
Neuron Degeneration ◽  
Sod1g93a Mouse ◽  
Lateral Sclerosis

Mapping of the motor neuron degeneration (Mnd) gene, a mouse model of amyotrophic lateral sclerosis (ALS)

Genomics ◽  
1992 ◽  
Vol 13 (3) ◽  
pp. 797-802 ◽  
Author(s):  
Anne Messer ◽  
Julie Plummer ◽  
Paul Maskin ◽  
John M. Coffin ◽  
Wayne N. Frankel
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Mouse Model ◽  
Motor Neuron ◽  
Motor Neuron Degeneration ◽  
Neuron Degeneration ◽  
Lateral Sclerosis

scholarly journals A microfluidic approach to rescue ALS motor neuron degeneration using rapamycin

2021 ◽  
Vol 11 (1) ◽  
Author(s):  
Phaneendra Chennampally ◽  
Ambreen Sayed-Zahid ◽  
Prabakaran Soundararajan ◽  
Jocelyn Sharp ◽  
Gregory A. Cox ◽  
...  
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Mouse Model ◽  
Motor Neuron ◽  
Motor Neurons ◽  
Embryonic Stem ◽  
Immunosuppressive Drug ◽  
Motor Neuron Degeneration ◽  
Neuron Degeneration ◽  
Lateral Sclerosis

AbstractTAR DNA-binding protein-43 (TDP-43) is known to accumulate in ubiquitinated inclusions of amyotrophic lateral sclerosis affected motor neurons, resulting in motor neuron degeneration, loss of motor functions, and eventually death. Rapamycin, an mTOR inhibitor and a commonly used immunosuppressive drug, has been shown to increase the survivability of Amyotrophic Lateral Sclerosis (ALS) affected motor neurons. Here we present a transgenic, TDP-43-A315T, mouse model expressing an ALS phenotype and demonstrate the presence of ubiquitinated cytoplasmic TDP-43 aggregates with > 80% cell death by 28 days post differentiation in vitro. Embryonic stem cells from this mouse model were used to study the onset, progression, and therapeutic remediation of TDP-43 aggregates using a novel microfluidic rapamycin concentration gradient generator. Results using a microfluidic device show that ALS affected motor neuron survival can be increased by 40.44% in a rapamycin dosage range between 0.4-1.0 µM.

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