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J Neuroimmunol. 2011 Jan;230(1-2):169-72. doi: 10.1016/j.jneuroim.2010.08.024.

Immunosuppression alters disease severity in juvenile Batten disease mice.

Author information

1
Center for Neural Development & Disease, University of Rochester School of Medicine & Dentistry, Rochester, NY, United States.

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.

PMID:
20937531
PMCID:
PMC3118572
DOI:
10.1016/j.jneuroim.2010.08.024
[Indexed for MEDLINE]
Free PMC Article

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