Progranulin does not affect motor neuron degeneration in mutant SOD1 mice and rats |
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Authors: | Sarah Herdewyn Louis De Muynck Ludo Van Den Bosch Wim Robberecht Philip Van Damme |
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Affiliation: | 1. Laboratory of Neurobiology, Experimental Neurology and Leuven Institute for Neurodegenerative Disorders (LIND) University of Leuven, Leuven, Belgium;2. Vesalius Research Center, VIB, Leuven, Belgium;3. Department of Neurology, University Hospital Leuven, University of Leuven, Leuven, Belgium |
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Abstract: | Motor neuron degeneration in amyotrophic lateral sclerosis (ALS) is familial in 10% of patients, with mutations in SOD1 and C9orf72 being the most frequent cause. There is convincing evidence for overlap between ALS and frontotemporal lobar degeneration at the genetic, pathological, and clinical level. Null mutations in progranulin (PGRN) are a frequent cause of familial frontotemporal lobar degeneration. PGRN exerts neurotrophic properties on motor neurons in vitro and in vivo. We therefore examined whether PGRN could affect disease progression in mutant SOD1 mice and rats, both established models for ALS. Overexpression of PGRN in mice and intracerebroventricular delivery of PGRN in rats did not affect onset or progression of motor neuron degeneration. |
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Keywords: | Frontotemporal lobar degeneration Amyotrophic lateral sclerosis Motor neuron degeneration Mutant superoxide dismutase 1 Progranulin |
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