Bi-phasic gliosis drives neuropathology in a Sandhoff disease mouse model.

Autor: Hooper AW; Department of Biology, McMaster University, Hamilton, Ont. L8S 4K1, Canada. Electronic address: hooperaw@Mcmaster.ca., Igdoura SA; Department of Biology, McMaster University, Hamilton, Ont. L8S 4K1, Canada; Department of Pathology and Molecular Medicine, McMaster University, Hamilton, Ont. L8S 4L8, Canada. Electronic address: igdoura@Mcmaster.ca.
Jazyk: angličtina
Zdroj: Journal of neuroimmunology [J Neuroimmunol] 2016 Oct 15; Vol. 299, pp. 19-27. Date of Electronic Publication: 2016 Aug 08.
DOI: 10.1016/j.jneuroim.2016.08.008
Abstrakt: Microgliosis and astrogliosis are known to be exacerbating factors in the progression of the lysosomal storage disorder Sandhoff disease. We have also found evidence for excitotoxicity via glutamate receptors in Sandhoff disease. To view the interaction of these cascades, we measured cerebellar expression of markers for gliosis, apoptosis, and excitatory synapses over the disease course in a Sandhoff disease mouse model. We observe a 2-stage model, with initial activation of microgliosis as early as 60days of age, followed by a later onset of astrogliosis, caspase-mediated apoptosis, and reduction in GluR1 at approximately 100days of age. These results implicate immune cells as first responders in Sandhoff disease.
(Copyright © 2016 Elsevier B.V. All rights reserved.)
Databáze: MEDLINE
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