Impairment of Neurite Formation in Familial ALS-associated Cu, Zn-Superoxide Dismutase Mutant Cells.
- Author:
Kyung Seok PARK
1
;
Hyun Jeong KIM
;
Man Ho KIM
;
Kwang Woo LEE
Author Information
1. Department of Neurology, Inje University Seoul Paik Hospital, Korea.
- Publication Type:Original Article
- Keywords:
Familial amyotrophic lateral sclerosis (ALS);
Mutations in Cu;
Zn-superoxide dismutase(SOD1);
Neural differentiation;
Neurite formation;
Neuronal death
- MeSH:
Amyotrophic Lateral Sclerosis;
Aphidicolin;
Blotting, Western;
Cell Death;
Humans;
Hybrid Cells;
Neurites*;
Neurons
- From:Journal of the Korean Neurological Association
2002;20(6):694-698
- CountryRepublic of Korea
- Language:Korean
-
Abstract:
BACKGROUND: Mutations in the human Cu, Zn-superoxide dismutase (SOD1) gene have been identified in some cases of familial amyotrophic lateral sclerosis (ALS). Neuronal cells with mutant SOD1 gene promoted cell death during differentiation by dibutyryl cAMP and aphidicolin. The aim of this study is to delineate if there is an impairment of the neural differentiation process in mutant SOD1 cells. METHODS: We studied the motoneuron-neuroblastoma hybrid cells (VSC 4.1) expressing wild-type or mutant SOD1 (G93A) during the differentiation by dibutyryl cAMP and aphidicolin. RESULTS: Mutant SOD1 cell (G93A) showed an impairment in the neurite formation. Western blot analysis revealed that the amount of neurofilament decreased before differentiation. A decrease in the amount of MAP-2 is observed during differentiation. CONCLUSIONS: Our results suggest that the impairment in the neurite formation of mutant SOD1 cell (G93A) is a differentiation failure and is associated with neuronal cell death.
