Literature DB >> 20937531

Immunosuppression alters disease severity in juvenile Batten disease mice.

Sabrina S Seehafer1, Denia Ramirez-Montealegre, Andrew Ms Wong, Chun-Hung Chan, Julian Castaneda, Michael Horak, Sarah M Ahmadi, Ming J Lim, Jonathan D Cooper, David A Pearce.   

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.
Copyright © 2010 Elsevier B.V. All rights reserved.

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Year:  2011        PMID： 20937531      PMCID： PMC3118572          DOI： 10.1016/j.jneuroim.2010.08.024

Source DB:  PubMed          Journal:  J Neuroimmunol        ISSN： 0165-5728            Impact factor:   3.478


  16 in total

1.  Distinct patterns of serum immunoreactivity as evidence for multiple brain-directed autoantibodies in juvenile neuronal ceroid lipofuscinosis.

Authors:  M J Lim; J Beake; E Bible; T M Curran; D Ramirez-Montealegre; D A Pearce; J D Cooper
Journal:  Neuropathol Appl Neurobiol       Date:  2006-10       Impact factor: 8.090

2.  Visual deficits in a mouse model of Batten disease are the result of optic nerve degeneration and loss of dorsal lateral geniculate thalamic neurons.

Authors:  Jill M Weimer; Andrew W Custer; Jared W Benedict; Noreen A Alexander; Evan Kingsley; Howard J Federoff; Jonathan D Cooper; David A Pearce
Journal:  Neurobiol Dis       Date:  2006-01-18       Impact factor: 5.996

3.  Autoimmunity to glutamic acid decarboxylase in the neurodegenerative disorder Batten disease.

Authors:  D Ramirez-Montealegre; S Chattopadhyay; T M Curran; C Wasserfall; L Pritchard; D Schatz; J Petitto; D Hopkins; J-X She; P G Rothberg; M Atkinson; D A Pearce
Journal:  Neurology       Date:  2005-02-22       Impact factor: 9.910

4.  The efficiency of systematic sampling in stereology and its prediction.

Authors:  H J Gundersen; E B Jensen
Journal:  J Microsc       Date:  1987-09       Impact factor: 1.758

Review 5.  Molecular genetics of the NCLs -- status and perspectives.

Authors:  Eija Siintola; Anna-Elina Lehesjoki; Sara E Mole
Journal:  Biochim Biophys Acta       Date:  2006-05-27

6.  Cerebellar defects in a mouse model of juvenile neuronal ceroid lipofuscinosis.

Authors:  Jill M Weimer; Jared W Benedict; Amanda L Getty; Charlie C Pontikis; Ming J Lim; Jonathan D Cooper; David A Pearce
Journal:  Brain Res       Date:  2009-02-20       Impact factor: 3.252

7.  IgG entry and deposition are components of the neuroimmune response in Batten disease.

Authors:  Ming J Lim; Noreen Alexander; Jared W Benedict; Subrata Chattopadhyay; Stephen J A Shemilt; Christopher J Guérin; Jonathan D Cooper; David A Pearce
Journal:  Neurobiol Dis       Date:  2006-10-27       Impact factor: 5.996

8.  A B cell-deficient mouse by targeted disruption of the membrane exon of the immunoglobulin mu chain gene.

Authors:  D Kitamura; J Roes; R Kühn; K Rajewsky
Journal:  Nature       Date:  1991-04-04       Impact factor: 49.962

9.  Attenuation of AMPA receptor activity improves motor skills in a mouse model of juvenile Batten disease.

Authors:  Attila D Kovács; David A Pearce
Journal:  Exp Neurol       Date:  2007-10-25       Impact factor: 5.330

10.  Identification of alpha-fetoprotein as an autoantigen in juvenile Batten disease.

Authors:  Julian A Castaneda; David A Pearce
Journal:  Neurobiol Dis       Date:  2007-08-25       Impact factor: 5.996
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  38 in total

1.  Females experience a more severe disease course in Batten disease.

Authors:  Jennifer Cialone; Heather Adams; Erika F Augustine; Frederick J Marshall; Jennifer M Kwon; Nicole Newhouse; Amy Vierhile; Erika Levy; Leon S Dure; Katherine R Rose; Denia Ramirez-Montealegre; Elisabeth A de Blieck; Jonathan W Mink
Journal:  J Inherit Metab Dis       Date:  2011-12-14       Impact factor: 4.982

2.  Prospects for stem cell therapy in neuronal ceroid lipofuscinosis.

Authors:  Michael C Kruer; David A Pearce; Paul J Orchard; Robert D Steiner
Journal:  Regen Med       Date:  2013-09       Impact factor: 3.806

3.  Progress toward Fulfilling the Potential of Immunomodulation in Childhood Neurodegeneration?

Authors:  Jonathan D Cooper; Hemanth R Nelvagal
Journal:  Mol Ther       Date:  2017-06-16       Impact factor: 11.454

4.  A canine model for neuronal ceroid lipofuscinosis highlights the promise of gene therapy for lysosomal storage diseases.

Authors:  Jonathan E Phillips; Richard H Gomer
Journal:  Ann Transl Med       Date:  2016-10

Review 5.  Clarifying lysosomal storage diseases.

Authors:  Mark L Schultz; Luis Tecedor; Michael Chang; Beverly L Davidson
Journal:  Trends Neurosci       Date:  2011-06-30       Impact factor: 13.837

6.  Using Patient-Specific Induced Pluripotent Stem Cells and Wild-Type Mice to Develop a Gene Augmentation-Based Strategy to Treat CLN3-Associated Retinal Degeneration.

Authors:  Luke A Wiley; Erin R Burnight; Arlene V Drack; Bailey B Banach; Dalyz Ochoa; Cathryn M Cranston; Robert A Madumba; Jade S East; Robert F Mullins; Edwin M Stone; Budd A Tucker
Journal:  Hum Gene Ther       Date:  2016-07-11       Impact factor: 5.695

7.  Antigen presenting cell abnormalities in the Cln3(-/-) mouse model of juvenile neuronal ceroid lipofuscinosis.

Authors:  Samantha L Hersrud; Attila D Kovács; David A Pearce
Journal:  Biochim Biophys Acta       Date:  2016-04-19

8.  A mixed breed dog with neuronal ceroid lipofuscinosis is homozygous for a CLN5 nonsense mutation previously identified in Border Collies and Australian Cattle Dogs.

Authors:  Natalie A Villani; Garrett Bullock; Jennifer R Michaels; Osamu Yamato; Dennis P O'Brien; Tendai Mhlanga-Mutangadura; Gary S Johnson; Martin L Katz
Journal:  Mol Genet Metab       Date:  2019-04-17       Impact factor: 4.797

Review 9.  Combination Therapies for Lysosomal Storage Diseases: A Complex Answer to a Simple Problem.

Authors:  Shannon L Macauley
Journal:  Pediatr Endocrinol Rev       Date:  2016-06

Review 10.  Vision loss in juvenile neuronal ceroid lipofuscinosis (CLN3 disease).

Authors:  Madhu M Ouseph; Mark E Kleinman; Qing Jun Wang
Journal:  Ann N Y Acad Sci       Date:  2016-01-08       Impact factor: 5.691
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