Title: Microglial upregulation of progranulin as a marker of motor neuron degeneration
Authors: Philips, Thomas
De Muynck, Louis
Nguyen, Thi Thu Hoai
Weynants, Beatrijs
Vanacker, Peter
Dhondt, Joke
Sleegers, Kristel
Schelhaas, Helenius J
Verbeek, Marcel
Vandenberghe, Rik
Sciot, Raphael
Van Broeckhoven, Christine
Lambrechts, Diether
Van Leuven, Freddy
Van Den Bosch, Ludo
Robberecht, Wim
Van Damme, Philip # ×
Issue Date: Dec-2010
Publisher: Association of the Journal of Neuropathology and Experimental Neurology
Series Title: Journal of Neuropathology and Experimental Neurology vol:69 issue:12 pages:1191-1200
Abstract: Frontotemporal lobar degeneration (FTLD) and amyotrophic lateral sclerosis (ALS) are overlapping neurodegenerative disorders. Mutations in the growth factor progranulin (PGRN) gene cause FTLD, sometimes in conjunction with ALS; such mutations are also observed in some ALS patients. Most PGRN mutations underlying FTLD are null mutations that result in reduced PGRN levels. We investigated PGRN expression in human ALS and in mouse models of motor neuron degeneration. Progranulin plasma or CSF levels in newly diagnosed ALS patients did not differ from those in healthy or disease controls (PGRN mutation-negative FTLD and Alzheimer disease patients). In the mutant SOD1 mouse model of ALS, spinal cord PGRN levels were normal in presymptomatic animals but increased during the degenerative process. This increase in PGRN correlated with enhanced expression of PGRN in microglia. In CSF, PGRN levels were normal in presymptomatic and early symptomatic animals, but with disease progression, a raise in PGRN was detectable. These data indicate that upregulation of PGRN is a marker of the microglial response that occurs with progression in motor neuron diseases.
ISSN: 0022-3069
Publication status: published
KU Leuven publication type: IT
Appears in Collections:Research Group Experimental Neurology
Laboratory for Neurobiology (Vesalius Research Center)
Vesalius Research Centre (-)
Translational Cell & Tissue Research
Department of Human Genetics - miscellaneous
Laboratory for Cognitive Neurology
Laboratory of Translational Genetics (Vesalius Research Center) (+)
× corresponding author
# (joint) last author

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