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Defective neurite outgrowth in aphidicolin/cAMP-induced motor neurons expressing mutant Cu/Zn superoxide dismutase
- Source :
-
International Journal of Developmental Neuroscience . Oct2002, Vol. 20 Issue 6, p521. 6p. - Publication Year :
- 2002
-
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. [Copyright &y& Elsevier]
- Subjects :
- *AMYOTROPHIC lateral sclerosis
*SUPEROXIDE dismutase
*APOPTOSIS
Subjects
Details
- Language :
- English
- ISSN :
- 07365748
- Volume :
- 20
- Issue :
- 6
- Database :
- Academic Search Index
- Journal :
- International Journal of Developmental Neuroscience
- Publication Type :
- Academic Journal
- Accession number :
- 7905270
- Full Text :
- https://doi.org/10.1016/S0736-5748(02)00052-7