Article
Detection method of the adjacent motor neuronal death in an in vitro co-culture model of familial ALS-associated Cu/Zn superoxide dismutase.
Biotechnology letters - 1 Aug 2004
Park Kyung-Seok, Kim Hyun-Jung, Choi Won-Jun, Kim Manho, Lee Kwang-Woo
Abstract excerpt
Mutations in Cu/Zn-superoxide dismutase (SOD1) gene have been identified in familial amyotrophic lateral sclerosis. Motor neuron degeneration subsequently spreads to contiguous neurons of the motor systems. We developed an in vitro disease model with motor neuron-neuroblastoma hybrid cells (VSC4.1) constitutively expressing a mutant (G93A) SOD1. The extracellular effect upon adjacent motor neurons was determined...
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