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

Abstract 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 cerebe...

Full description

Saved in:
Bibliographic Details
Published in:Journal of neuroimmunology 2016-10, Vol.299, p.19-27
Main Authors: Hooper, Alexander W.M, Igdoura, Suleiman
Format: Article
Language:English
Subjects:
Allergy and Immunology
Animals
Apoptosis
Astrogliosis
beta-Hexosaminidase beta Chain - genetics
Cerebellum - metabolism
Cerebellum - pathology
Cleaved-caspase 9
Disease Models, Animal
Female
Gliosis - genetics
Gliosis - metabolism
Gliosis - pathology
Glur1
Mice
Mice, Inbred C57BL
Mice, Knockout
Microgliosis
Neurology
Sandhoff disease
Sandhoff Disease - genetics
Sandhoff Disease - metabolism
Sandhoff Disease - pathology
Citations: Items that this one cites
Items that cite this one
Online Access:Get full text
Tags: Add Tag
No Tags, Be the first to tag this record!
Description
Summary:Abstract 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 60 days of age, followed by a later onset of astrogliosis, caspase-mediated apoptosis, and reduction in GluR1 at approximately 100 days of age. These results implicate immune cells as first responders in Sandhoff disease.
ISSN:0165-5728
1872-8421
DOI:10.1016/j.jneuroim.2016.08.008