J Korean Neurol Assoc > Volume 19(6); 2001 > Article

Journal of the Korean Neurological Association 2001;19(6): 629-632.

변이 SOD-1 유전자 발현 운동신경세포 배양을 이용한 가족성 근위축성측삭경화증의 세포외 독성

최원준, 김현정 전종은 박경석 김성훈 김만호 이광우

서울대학교 신경과학교실

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

"Won-Jun Choi, M.D., Hyun Jeong Kim, B.S., Jong-Un Chun, M.D., Kyung-Seok Park, M.D., Sung-Hoon Kim, M.D., Manho Kim, M.D., Kwang-Woo Lee, M.D., Ph.D."

Department of Neurology, Seoul National University Hospital

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. Key Words : Amyotrophic lateral sclerosis, Cytotoxicity, Cell communication, Motor neurons"