AAV-mediated gene delivery attenuates neuroinflammation in feline Sandhoff disease

Highlights • Neuroinflammation is a prominent pathological feature of feline Sandhoff disease. • A robust infiltration and activation of microglia were evident in CNS of SD cats. • MHCII and MIP-1α were up-regulated in the brain of SD cats. • Intracranial AAV gene therapy attenuated inflammation, no...

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Bibliographic Details
Published in:Neuroscience 2017-01, Vol.340, p.117-125
Main Authors: Bradbury, Allison M, Peterson, Tiffany A, Gross, Amanda L, Wells, Stephen Z, McCurdy, Victoria J, Wolfe, Karen G, Dennis, John C, Brunson, Brandon L, Gray-Edwards, Heather, Randle, Ashley N, Johnson, Aime K, Morrison, Edward E, Cox, Nancy R, Baker, Henry J, Sena-Esteves, Miguel, Martin, Douglas R
Format: Article
Language:English
Subjects:
AAV gene therapy
Adaptor Proteins, Signal Transducing - metabolism
Adeno-associated virus
Animals
Astrocytes - immunology
Astrocytes - pathology
Brain - immunology
Brain - pathology
Cats
cytokines/chemokines
Dependovirus - genetics
Disease Models, Animal
Genes, MHC Class II - physiology
Genetic Therapy
Genetic Vectors
Gliosis - immunology
Gliosis - pathology
Gliosis - therapy
Immunohistochemistry
lysosomal storage disorder
Microglia - immunology
Microglia - pathology
microglia activation
neurodegenerative disease
neuroinflammation
Neurology
Neurons - immunology
Neurons - pathology
Polymerase Chain Reaction
Sandhoff Disease - immunology
Sandhoff Disease - pathology
Sandhoff Disease - therapy
Transcription Factors - genetics
Transcription Factors - metabolism
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Summary:Highlights • Neuroinflammation is a prominent pathological feature of feline Sandhoff disease. • A robust infiltration and activation of microglia were evident in CNS of SD cats. • MHCII and MIP-1α were up-regulated in the brain of SD cats. • Intracranial AAV gene therapy attenuated inflammation, normalizing MHCII and MIP-1α.
ISSN:0306-4522
1873-7544
DOI:10.1016/j.neuroscience.2016.10.047