Models of amyotrophic lateral sclerosis.
Amyotrophic lateral sclerosis (ALS) is a progressive neurological disorder caused by degeneration of the motor neurons in cortex, brainstem and spinal cord. Two experimental models of ALS are described in this unit: organotypic cultures of spinal cord, and transgenic mice expressing a human mutant superoxide dismutase 1 (SOD1) gene. Appropriate animal and cell culture models of ALS can be used to help unravel the sequence of events in motor neuronal degeneration and test potential therapies.
Jackson
Mandy
M
Johns Hopkins University, Baltimore, Maryland, USA.
Ganel
Raquelli
R
Rothstein
Jeffrey D
JD
D000690:Amyotrophic Lateral Sclerosis / Q000201:enzymology / Q000235:genetics* / Q000503:physiopathology; D000818:Animals; D004195:Disease Models, Animal; D006801:Humans; D051379:Mice; D008822:Mice, Transgenic; D009924:Organ Culture Techniques / Q000379:methods; D051381:Rats