J Korean Neurol Assoc.  2002 Nov;20(6):694-698.

Impairment of Neurite Formation in Familial ALS-associated Cu, Zn-Superoxide Dismutase Mutant Cells

• Park KS
• Kim HJ
• Kim MH
• Lee KW

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.

Keyword

Familial amyotrophic lateral sclerosis (ALS); Mutations in Cu; Zn-superoxide dismutase(SOD1); Neural differentiation; Neurite formation; Neuronal death

MeSH Terms

Amyotrophic Lateral Sclerosis
Aphidicolin
Blotting, Western
Cell Death
Humans
Hybrid Cells
Neurites*
Neurons
Aphidicolin