title: Progranulin does not affect motor neuron degeneration in mutant SOD1 mice and rats.
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.
authors.affiliation: Laboratory of Neurobiology, Experimental Neurology and Leuven Institute for Neurodegenerative Disorders (LIND) University of Leuven, Leuven, Belgium.
authors.forename: Sarah
authors.initials: S
authors.lastname: Herdewyn
authors.forename: Louis
authors.initials: L
authors.lastname: De Muynck
authors.forename: Ludo
authors.initials: L
authors.lastname: Van Den Bosch
authors.forename: Wim
authors.initials: W
authors.lastname: Robberecht
authors.forename: Philip
authors.initials: P
authors.lastname: Van Damme
mesh_terms: D000690:Amyotrophic Lateral Sclerosis / Q000235:genetics* / Q000473:pathology*; D000818:Animals; D000073885:C9orf72 Protein; D004195:Disease Models, Animal; D018450:Disease Progression; D057174:Frontotemporal Lobar Degeneration / Q000235:genetics; D015870:Gene Expression; D000077155:Granulins; D036341:Intercellular Signaling Peptides and Proteins / Q000235:genetics* / Q000502:physiology; D051379:Mice; D008817:Mice, Mutant Strains; D009046:Motor Neurons / Q000473:pathology*; D009154:Mutation; D009410:Nerve Degeneration; D000077153:Progranulins; D011506:Proteins / Q000235:genetics; D051381:Rats; D011922:Rats, Mutant Strains; D013482:Superoxide Dismutase / Q000235:genetics*; D000072105:Superoxide Dismutase-1
chemical_list: D000073885:C9orf72 Protein; C568651:C9orf72 protein, human; D000077155:Granulins; C120204:Grn protein, mouse; D036341:Intercellular Signaling Peptides and Proteins; D000077153:Progranulins; D011506:Proteins; C000606290:SOD1 protein, human; C000606291:Sod1 protein, mouse; C000606292:Sod1 protein, rat; D013482:Superoxide Dismutase; D000072105:Superoxide Dismutase-1
keywords: Amyotrophic lateral sclerosis; Frontotemporal lobar degeneration; Motor neuron degeneration; Mutant superoxide dismutase 1; Progranulin