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Neurobiol Aging. 2013 Nov;34(11):2548-50. doi: 10.1016/j.neurobiolaging.2013.04.019. Epub 2013 May 21.

Sensitivity to neurotoxic stress is not increased in progranulin-deficient mice.

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Centre for Molecular Medicine & Therapeutics, Department of Medical Genetics, University of British Columbia, and Children's and Women's Hospital, 980 West 28th Avenue, Vancouver, BC, Canada V5Z 4H4.


Loss-of-function mutations in the progranulin (GRN) gene are a common cause of autosomal dominant frontotemporal lobar degeneration, a fatal and progressive neurodegenerative disorder common in people less than 65 years of age. In the brain, progranulin is expressed in multiple regions at varying levels, and has been hypothesized to play a neuroprotective or neurotrophic role. Four neurotoxic agents were injected in vivo into constitutive progranulin knockout (Grn(-/-)) mice and their wild-type (Grn(+/+)) counterparts to assess neuronal sensitivity to toxic stress. Administration of 3-nitropropionic acid, quinolinic acid, kainic acid, and pilocarpine induced robust and measurable neuronal cell death in affected brain regions, but no differential cell death was observed between Grn(+/+) and Grn(-/-) mice. Thus, constitutive progranulin knockout mice do not have increased sensitivity to neuronal cell death induced by the acute chemical models of neuronal injury used in this study.


3-Nitropropionic acid; Frontotemporal dementia; Frontotemporal lobar degeneration; Kainic acid; Neurotoxin; Pilocarpine; Progranulin; Progranulin knockout mouse; Quinolinic acid

[Indexed for MEDLINE]

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