Article
Human embryonic stem cell-derived motor neurons expressing SOD1 mutants exhibit typical signs of motor neuron degeneration linked to ALS.
Disease models & mechanisms - 1 Jan 2009
Karumbayaram Saravanan, Kelly Theresa K, Paucar Andres A, Roe Anne J T, Umbach Joy A, Charles Andrew, Goldman Steven A, Kornblum Harley I, Wiedau-Pazos Martina
Abstract excerpt
Human embryonic stem cell (hESC)-derived neurons have the potential to model neurodegenerative disorders. Here, we demonstrate the expression of a mutant gene, superoxide dismutase 1(SOD1), linked to familial amyotrophic lateral sclerosis (ALS) in hESC-derived motor neurons. Green fluorescent protein (GFP) expression under the control of the HB9 enhancer was used to identify SOD1-transfected motor neurons that...
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