J Korean Neurol Assoc.  2001 Nov;19(6):629-632.

Extracellular Toxicity of Motor Neuronal Cells Expressing Mutant Cu/Zn Superoxide Dismutase in Familial ALS Cell Line Model

• Choi WJ
• Kim HJ
• Chun JU
• Park KS
• Kim SH
• Kim MH
• Lee KW

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.

Keyword

Amyotrophic lateral sclerosis; Cytotoxicity; Cell communication; Motor neurons

MeSH Terms

Amyotrophic Lateral Sclerosis
Cell Communication
Cell Line*
Cell Survival
Coculture Techniques
Humans
Motor Neurons*
Plasmids
Superoxide Dismutase*
Superoxides*
Superoxide Dismutase
Superoxides