Immunosuppression alters disease severity in juvenile Batten disease mice

Sabrina S. Seehafer, Denia Ramirez-Montealegre, Andrew M.S. Wong, Chun Hung Chan, Julian Castaneda, Michael Horak, Sarah M. Ahmadi, Ming J. Lim, Jonathan D. Cooper, David A. Pearce

Research output: Contribution to journalArticlepeer-review

69 Scopus citations

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.

Original languageEnglish
Pages (from-to)169-172
Number of pages4
JournalJournal of Neuroimmunology
Volume230
Issue number1-2
DOIs
StatePublished - Jan 2011

Keywords

  • Batten disease
  • CLN3
  • Immunosuppression
  • JNCL

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