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

doi:10.1016/j.jneuroim.2010.08.024

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 journal › Article › peer-review

73 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 language	English
Pages (from-to)	169-172
Number of pages	4
Journal	Journal of Neuroimmunology
Volume	230
Issue number	1-2
DOIs	https://doi.org/10.1016/j.jneuroim.2010.08.024
State	Published - Jan 2011

Keywords

Batten disease
CLN3
Immunosuppression
JNCL

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10.1016/j.jneuroim.2010.08.024

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title = "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 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.",

keywords = "Batten disease, CLN3, Immunosuppression, JNCL",

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

year = "2011",

month = jan,

doi = "10.1016/j.jneuroim.2010.08.024",

language = "English",

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journal = "Journal of Neuroimmunology",

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AU - Seehafer, Sabrina S.

AU - Ramirez-Montealegre, Denia

AU - Wong, Andrew M.S.

AU - Chan, Chun Hung

AU - Castaneda, Julian

AU - Horak, Michael

AU - Ahmadi, Sarah M.

AU - Lim, Ming J.

AU - Cooper, Jonathan D.

AU - Pearce, David A.

PY - 2011/1

Y1 - 2011/1

N2 - 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.

AB - 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.

KW - Batten disease

KW - CLN3

KW - Immunosuppression

KW - JNCL

UR - https://www.scopus.com/pages/publications/78650993597

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DO - 10.1016/j.jneuroim.2010.08.024

M3 - Article

C2 - 20937531

AN - SCOPUS:78650993597

SN - 0165-5728

VL - 230

SP - 169

EP - 172

JO - Journal of Neuroimmunology

JF - Journal of Neuroimmunology

IS - 1-2

ER -

Immunosuppression alters disease severity in juvenile Batten disease mice

Abstract

Keywords

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