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- Title
Defective neurite outgrowth in aphidicolin/cAMP-induced motor neurons expressing mutant Cu/Zn superoxide dismutase
- Authors
Lee, Kwang-Woo; Kim, Hyun-Jeong; Sung, Jung-Joon; Park, Kyung-Seok; Kim, Manho
- Abstract
Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disorder characterized by motor neuron involvement. Mutations in the human Cu/Zn superoxide dismutase (SOD1) gene are found in some cases of familial ALS. Many studies have reported SOD1 mutation-related neurodegeneration. However, whether or not a mutant SOD1 affects neural development has not been demonstrated. We developed motor neuron-neuroblastoma hybrid cells that expressed a mutant (G93A) or the wild type (WT) SOD1. Cells were differentiated by dibutyryl cAMP and aphidicolin. The mutant showed a defect in neurite outgrowth and had decreased viability. Cytochrome c released and nuclear fragmentation were observed. Western blot analysis showed that the amount of neurofilament and microtubule associated proteins-2 (MAP-2) decreased during differentiation. These results suggest that the defect in neurite outgrowth of mutant SOD1 cells is a cytoskeletal defect and is associated with neuronal death.
- Subjects
AMYOTROPHIC lateral sclerosis; SUPEROXIDE dismutase; APOPTOSIS
- Publication
International Journal of Developmental Neuroscience, 2002, Vol 20, Issue 6, p521
- ISSN
0736-5748
- Publication type
Article
- DOI
10.1016/S0736-5748(02)00052-7