Extracellular Toxicity of Motor Neuronal Cells Expressing Mutant Cu/Zn Superoxide Dismutase in Familial ALS Cell Line Model.
- Author:
Won Jun CHOI
1
;
Hyun Jeong KIM
;
Jong Un CHUN
;
Kyung Seok PARK
;
Sung Hoon KIM
;
Man Ho KIM
;
Kwang Woo LEE
Author Information
1. Department of Neurology, Seoul National University Hospital, Korea. kimmanho@snu.ac.
- Publication Type:Original Article
- Keywords:
Amyotrophic lateral sclerosis;
Cytotoxicity;
Cell communication;
Motor neurons
- MeSH:
Amyotrophic Lateral Sclerosis;
Cell Communication;
Cell Line*;
Cell Survival;
Coculture Techniques;
Humans;
Motor Neurons*;
Plasmids;
Superoxide Dismutase*;
Superoxides*
- From:Journal of the Korean Neurological Association
2001;19(6):629-632
- CountryRepublic of Korea
- Language:Korean
-
Abstract:
BACKGROUND: The objective of this study is to identify the extracellular toxicity of motor neuronal cells expressing mutant copper-zinc superoxide dismutase in the model of familial amyotrophic lateral sclerosis (FALS), and to investi-gate their possible mechanisms in motor neuron death. METHODS: We have set up a model for FALS by transfecting the motor neuron cell line VSC4.1 with plasmids directing the constitutive expression of either wild-type human Cu/Zn superoxide dismutase or a mutant of this enzyme, G93A. The co-culture model of motor neuronal cells expressing both mutant and wild-type Cu/Zn superoxide dismutases were used. Cell toxicity was induced by aphidocholin and viability was determined by a MTT assay. The observed values were compared with predictive values in G93A+VSC4.1 as well as WT+VSC4.1 co-culture groups. RESULTS: In the co-culture group with G93A and VSC4.1, the observed cell viability was significantly lower than what was predicted, suggesting that the G93A affected the viability of VSC4.1. However, in the co-culture group with WT and VSC4.1, WT did not decrease the viability of VSC4.1. CONCLUSIONS: The G93A cells have extracellular toxicity, which could be a result of some kind of cell-to-cell communications between motor neuronal cells.
