Amyotrophic lateral sclerosis (ALS) is a degenerative motor neuron disease which currently has no cure. Research using rodent ALS models transgenic for mutant superoxide dismutase 1 (SOD1) has implicated that glial-neuronal interactions play a major role in the destruction of motor neurons, but the generality of this mechanism is not clear as SOD1 mutations only account for less than 2% of all ALS cases. Recently, this hypothesis was backed up by observation of similar effects using astrocytes derived from post-mortem spinal cord tissue of ALS patients which did not carry SOD1 mutations. However, such necropsy samples may not be easy to obtain and may not always yield viable cell cultures. Here, we have analysed olfactory mucosa (OM) cells, which can be easily isolated from living ALS patients. Disease-specific changes observed when ALS OM cells were co-cultured with human spinal cord neurons included decreased neuronal viability, aberrant neuronal morphology and altered glial inflammatory responses. Our results show the potential of OM cells as new cell models for ALS.
J Cell Mol Med
1284 - 1295
SOD-1 neurotoxicity, amyotrophic lateral sclerosis, inflammation-responsive promoter, non-cell autonomous toxicity, olfactory mucosa, Amyotrophic Lateral Sclerosis, Astrocytes, Blotting, Western, Cell Survival, Cells, Cultured, Coculture Techniques, Glial Fibrillary Acidic Protein, Humans, Immunohistochemistry, Microscopy, Fluorescence, Models, Biological, Mutation, Neurons, Olfactory Mucosa, Primary Cell Culture, Spinal Cord, Superoxide Dismutase, Superoxide Dismutase-1, Vimentin