Immunosuppression alters disease severity in juvenile Batten disease mice

Abstract Autoantibodies to brain proteins are present in Juvenile Neuronal Ceroid Lipofuscinosis (Batten disease) patients and in the Cln3 −/− mouse model of this disease, suggesting an autoimmune component to pathogenesis. Using genetic or pharmaceutical approaches to attenuate this immune response...

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Bibliographic Details
Published in:Journal of neuroimmunology 2011-01, Vol.230 (1), p.169-172
Main Authors: Seehafer, Sabrina S, Ramirez-Montealegre, Denia, Wong, Andrew MS, Chan, Chun-Hung, Castaneda, Julian, Horak, Michael, Ahmadi, Sarah M, Lim, Ming J, Cooper, Jonathan D, Pearce, David A
Format: Article
Language:English
Subjects:
Allergy and Immunology
Animals
Autoantibodies - drug effects
Autoantibodies - immunology
Batten disease
Blotting, Western
Brain - drug effects
Brain - pathology
Cell Count
CLN3
Disease Models, Animal
Immunohistochemistry
Immunosuppression
Immunosuppressive Agents - pharmacology
JNCL
Male
Membrane Glycoproteins - genetics
Mice
Mice, Inbred C57BL
Mice, Knockout
Molecular Chaperones - genetics
Motor Skills - drug effects
Mycophenolic Acid - analogs & derivatives
Mycophenolic Acid - pharmacology
Neurology
Neuronal Ceroid-Lipofuscinoses - drug therapy
Neuronal Ceroid-Lipofuscinoses - immunology
Neuronal Ceroid-Lipofuscinoses - pathology
Neurons - drug effects
Neurons - pathology
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Summary:Abstract Autoantibodies to brain proteins are present in Juvenile Neuronal Ceroid Lipofuscinosis (Batten disease) patients and in the Cln3 −/− mouse model of this disease, suggesting an autoimmune component to pathogenesis. Using genetic or pharmaceutical approaches to attenuate this immune response in Cln3 −/− mice, we demonstrate decreased neuroinflammation, decreased deposition of immunoglobulin G in the brain and protection of vulnerable neuron populations. Moreover, immune suppression results in a significant improvement in motor performance providing for the first plausible therapeutic approach for juvenile Batten disease.
ISSN:0165-5728
1872-8421
1872-8421
DOI:10.1016/j.jneuroim.2010.08.024