J Korean Neurol Assoc > Volume 20(5); 2002 > Article

Journal of the Korean Neurological Association 2002;20(5): 525-529.

Dismutase 유전자를 발현하는 운동신경세포의 세포생존율에 미치는 영향

김한준 , 박성호 ·김만호 ·김현정 ·이광우

서울대학교 의과대학 신경과학교실,서울특별시립 보라매병원 신경과학교실

"Neuroprotective Effect of Estrogen on Motor Neuron Cells Expressing Wild Type or Mutant Cu/Zn Superoxide Dismutase"

Han-Joon Kim, M.D.

"Department of Neurology, College of Medicine, Seoul National University, Department of Neurology, Seoul Municipal Boramae Hospital"

Abstract

"Background : Recently, the mechanism involved in nitric oxide (NO)-mediated motor neuron death is under exten-sive investigation. The role of Cu/Zn superoxide dismutase (SOD) mutation, which is found in about 2% of all ALS patients, has been implicated in selective motor neuron death and it is said to play an important role in NO-mediated motor neuron death. Estrogen is reported to have neuroprotective effect in various neurological diseases. However, neu-roprotective effect on estradiol on spinal motor neuron exposed to NO has rarely been studied. Methods : Motor neu-ron- neuroblastoma hybrid cell expressing wild-type or mutant (G93A or A4V) SOD gene was treated with 200 μM S-nitrosoglutathione. After 24 hours, cell viability was measured by MTT assay. To see the neuroprotective effect of estradiol, pretreatment with 5 nM or 50 nM 17 β-estradiols was done 24 hours before S-nitrosoglutathione treatment. Results : S-nitrosoglutathione showed significant neurotoxic effect in all three cell lines. Percentage of cell death was significantly different in each cell line. Both 5 nM and 50 nM estradiols showed neuroprotective effect in G93A cell line. In wild-type cell line, 50 nM estradiol showed neuroprotective effect, but 5 nM estradiol did not. In A4V cell line, estradiol did not showed neuroprotective effect. Conclusions : This study showed that NO-mediated motor neuron death could be influenced by presence or absence of mutation and type of mutation in SOD gene. Neuroprotective effect of estradiol is also influenced by SOD gene mutation. This study implies that estrogen might be beneficial to some ALS patients.Key words : Amyotrophic lateral sclerosis (ALS), Cell viability, Neuroprotection, Cu/Zn superoxide dismutase (SOD),"