Patient‐derived olfactory mucosa for study of the non‐neuronal contribution to amyotrophic lateral sclerosis pathology |
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Authors: | Vega García‐Escudero María Rosales José Luis Muñoz Esteban Scola Javier Medina Hena Khalique Guillermo Garaulet Antonio Rodriguez Filip Lim |
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Institution: | 1. Departamento de Biología Molecular, Universidad Autónoma de Madrid, Madrid, Spain;2. Centro de Biología Molecular “Severo Ochoa” (C.S.I.C.‐ U.A.M.), Universidad Autónoma de Madrid, Madrid, Spain;3. Departamento de Neurología, Hospital General Universitario Gregorio Mara?ón, Madrid, Spain;4. Departamento de Otorrinolaringología, Hospital General Universitario Gregorio Mara?ón, Madrid, Spain |
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Abstract: | 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. |
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Keywords: | olfactory mucosa amyotrophic lateral sclerosis non‐cell autonomous toxicity SOD‐1 neurotoxicity inflammation‐responsive promoter |
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