Immunosuppression alters disease severity in juvenile Batten disease mice

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• 作者：Sabrina S. Seehafer^a ; Denia Ramirez-Montealegre^a ; Andrew MS Wong^b ; Chun-Hung Chan^a ; ¹ ; Julian Castaneda^a ; Michael Horak^b ; Sarah M. Ahmadi^b ; Ming J. Lim^b ; Jonathan D. Cooper^b ; David A. Pearce^a ; ^c ; ^d ; ¹ ; ^{David.Pearce@sanfordhealth.org}
• 关键词：CLN3 ; JNCL ; Batten disease ; Immunosuppression
• 刊名：Journal of Neuroimmunology
• 出版年：2011
• 出版时间：January 2011
• 年：2011
• 卷：230
• 期：1-2
• 页码：169-172
• 全文大小：807 K

文摘

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.