scholarly journals Human embryonic stem cell-derived motor neurons expressing SOD1 mutants exhibit typical signs of motor neuron degeneration linked to ALS

2009 ◽  
Vol 2 (3-4) ◽  
pp. 189-195 ◽  
Author(s):  
S. Karumbayaram ◽  
T. K. Kelly ◽  
A. A. Paucar ◽  
A. J. T. Roe ◽  
J. A. Umbach ◽  
...  
Keyword(s):  
Stem Cell ◽  
Embryonic Stem Cell ◽  
Motor Neuron ◽  
Motor Neurons ◽  
Human Embryonic Stem Cell ◽  
Embryonic Stem ◽  
Motor Neuron Degeneration ◽  
Neuron Degeneration

scholarly journals Human Embryonic Stem Cell-Derived Motor Neurons Are Sensitive to the Toxic Effect of Glial Cells Carrying an ALS-Causing Mutation

2008 ◽  
Vol 3 (6) ◽  
pp. 637-648 ◽  
Author(s):  
Francesco Paolo Di Giorgio ◽  
Gabriella L. Boulting ◽  
Samuel Bobrowicz ◽  
Kevin C. Eggan
Keyword(s):  
Stem Cell ◽  
Embryonic Stem Cell ◽  
Toxic Effect ◽  
Glial Cells ◽  
Motor Neurons ◽  
Human Embryonic Stem Cell ◽  
Embryonic Stem

Enrichment of human embryonic stem cell derived motor neuron cultures using arabinofuranosyl cytidine

2015 ◽  
Vol 10 (2) ◽  
pp. 91-99
Author(s):  
Ava Masoumi ◽  
Eric E Low ◽  
Tarannom Shoghi ◽  
Pik K Chan ◽  
Chie F Hsiao ◽  
...  
Keyword(s):  
Stem Cell ◽  
Embryonic Stem Cell ◽  
Motor Neuron ◽  
Human Embryonic Stem Cell ◽  
Embryonic Stem

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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