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Publication : Progranulin does not affect motor neuron degeneration in mutant SOD1 mice and rats.

First Author  Herdewyn S Year  2013
Journal  Neurobiol Aging Volume  34
Issue  10 Pages  2302-3
PubMed ID  23608112 Mgi Jnum  J:211734
Mgi Id  MGI:5576100 Doi  10.1016/j.neurobiolaging.2013.03.027
Citation  Herdewyn S, et al. (2013) Progranulin does not affect motor neuron degeneration in mutant SOD1 mice and rats. Neurobiol Aging 34(10):2302-3
abstractText  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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