Late onset neurodegeneration in the Cln3−/− mouse model of juvenile neuronal ceroid lipofuscinosis is preceded by low level glial activation

Charlie C. Pontikis; Claire V. Cella; Nisha Parihar; Ming J. Lim; Shubhodeep Chakrabarti; Hannah M. Mitchison; William C. Mobley; Payam Rezaie; David A. Pearce; Jonathan D. Cooper

doi:10.1016/j.brainres.2004.07.030

Late onset neurodegeneration in the Cln3−/− mouse model of juvenile neuronal ceroid lipofuscinosis is preceded by low level glial activation

Brain Research ◽

10.1016/j.brainres.2004.07.030 ◽

2004 ◽

Vol 1023 (2) ◽

pp. 231-242 ◽

Cited By ~ 109

Author(s):

Charlie C. Pontikis ◽

Claire V. Cella ◽

Nisha Parihar ◽

Ming J. Lim ◽

Shubhodeep Chakrabarti ◽

...

Keyword(s):

Mouse Model ◽

Late Onset ◽

Neuronal Ceroid Lipofuscinosis ◽

Glial Activation ◽

Juvenile Neuronal Ceroid Lipofuscinosis ◽

Low Level ◽

Ceroid Lipofuscinosis

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Altered Flurothyl Seizure Induction Latency, Phenotype, and Subsequent Mortality in a Mouse Model of Juvenile Neuronal Ceroid Lipofuscinosis/Batten Disease

Epilepsia ◽

10.1046/j.1528-1157.2002.16002.x ◽

2002 ◽

Vol 43 (10) ◽

pp. 1137-1140 ◽

Cited By ~ 11

Author(s):

Elizabeth Kriscenski-Perry ◽

Craig D. Applegate ◽

Andrew Serour ◽

Timothy R. Mhyre ◽

Christopher C. Leonardo ◽

...

Keyword(s):

Mouse Model ◽

Neuronal Ceroid Lipofuscinosis ◽

Batten Disease ◽

Juvenile Neuronal Ceroid Lipofuscinosis ◽

Ceroid Lipofuscinosis

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Juvenile neuronal ceroid lipofuscinosis (JNCL) is associated with aberrant glial activation in the central nervous system

Molecular Genetics and Metabolism ◽

10.1016/j.ymgme.2012.11.127 ◽

2013 ◽

Vol 108 (2) ◽

pp. S53

Author(s):

Tammy Kielian ◽

Maria Burkovetskaya ◽

Nikolay Karpuk ◽

Juan Xiong

Keyword(s):

Central Nervous System ◽

Nervous System ◽

Neuronal Ceroid Lipofuscinosis ◽

Glial Activation ◽

Juvenile Neuronal Ceroid Lipofuscinosis ◽

Ceroid Lipofuscinosis ◽

The Central Nervous System

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Partial Correction of the CNS Lysosomal Storage Defect in a Mouse Model of Juvenile Neuronal Ceroid Lipofuscinosis by Neonatal CNS Administration of an Adeno-Associated Virus Serotype rh.10 Vector Expressing the Human CLN3 Gene

Human Gene Therapy ◽

10.1089/hum.2012.253 ◽

2014 ◽

Vol 25 (3) ◽

pp. 223-239 ◽

Cited By ~ 25

Author(s):

Dolan Sondhi ◽

Emma C. Scott ◽

Alvin Chen ◽

Neil R. Hackett ◽

Andrew M.S. Wong ◽

...

Keyword(s):

Mouse Model ◽

Neuronal Ceroid Lipofuscinosis ◽

Lysosomal Storage ◽

Adeno Associated Virus ◽

Juvenile Neuronal Ceroid Lipofuscinosis ◽

Ceroid Lipofuscinosis ◽

Virus Serotype ◽

Partial Correction

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Author response: Defective synaptic transmission causes disease signs in a mouse model of juvenile neuronal ceroid lipofuscinosis

10.7554/elife.28685.027 ◽

2017 ◽

Author(s):

Benedikt Grünewald ◽

Maren D Lange ◽

Christian Werner ◽

Aet O'Leary ◽

Andreas Weishaupt ◽

...

Keyword(s):

Mouse Model ◽

Synaptic Transmission ◽

Neuronal Ceroid Lipofuscinosis ◽

Author Response ◽

Juvenile Neuronal Ceroid Lipofuscinosis ◽

Ceroid Lipofuscinosis

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Altered sensitivity of cerebellar granule cells to glutamate receptor overactivation in the Cln3Δex7/8-knock-in mouse model of juvenile neuronal ceroid lipofuscinosis

Neurochemistry International ◽

10.1016/j.neuint.2011.02.003 ◽

2011 ◽

Vol 58 (6) ◽

pp. 648-655 ◽

Cited By ~ 25

Author(s):

Rozzy Finn ◽

Attila D. Kovács ◽

David A. Pearce

Keyword(s):

Mouse Model ◽

Glutamate Receptor ◽

Granule Cells ◽

Cerebellar Granule Cells ◽

Neuronal Ceroid Lipofuscinosis ◽

Juvenile Neuronal Ceroid Lipofuscinosis ◽

Cerebellar Granule ◽

Ceroid Lipofuscinosis ◽

Altered Sensitivity

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Late onset juvenile neuronal ceroid-lipofuscinosis with granular osmiophilic deposits (GROD)

American Journal of Medical Genetics ◽

10.1002/ajmg.1320570211 ◽

1995 ◽

Vol 57 (2) ◽

pp. 165-167 ◽

Cited By ~ 13

Author(s):

Irene L. Hofman ◽

Peter E. M. Taschner

Keyword(s):

Late Onset ◽

Neuronal Ceroid Lipofuscinosis ◽

Juvenile Neuronal Ceroid Lipofuscinosis ◽

Ceroid Lipofuscinosis

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Retinal Pathology and Function in a Cln3 Knockout Mouse Model of Juvenile Neuronal Ceroid Lipofuscinosis (Batten Disease)

Molecular and Cellular Neuroscience ◽

10.1006/mcne.2001.1099 ◽

2002 ◽

Vol 19 (4) ◽

pp. 515-527 ◽

Cited By ~ 38

Author(s):

Gail M. Seigel ◽

Andrew Lotery ◽

Ari Kummer ◽

David J. Bernard ◽

Nicholas D.E. Greene ◽

...

Keyword(s):

Mouse Model ◽

Knockout Mouse ◽

Neuronal Ceroid Lipofuscinosis ◽

Batten Disease ◽

Juvenile Neuronal Ceroid Lipofuscinosis ◽

Knockout Mouse Model ◽

Ceroid Lipofuscinosis ◽

Retinal Pathology ◽

And Function

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Phenotypic characterization of a mouse model of juvenile neuronal ceroid lipofuscinosis

Neurobiology of Disease ◽

10.1016/j.nbd.2007.08.017 ◽

2008 ◽

Vol 29 (2) ◽

pp. 242-253 ◽

Cited By ~ 24

Author(s):

Martin L. Katz ◽

Gary S. Johnson ◽

Gregory E. Tullis ◽

Bo Lei

Keyword(s):

Mouse Model ◽

Neuronal Ceroid Lipofuscinosis ◽

Phenotypic Characterization ◽

Juvenile Neuronal Ceroid Lipofuscinosis ◽

Ceroid Lipofuscinosis

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Progressive Retinal Degeneration and Glial Activation in the CLN6nclf Mouse Model of Neuronal Ceroid Lipofuscinosis: A Beneficial Effect of DHA and Curcumin Supplementation

PLoS ONE ◽

10.1371/journal.pone.0075963 ◽

2013 ◽

Vol 8 (10) ◽

pp. e75963 ◽

Cited By ~ 35

Author(s):

Myriam Mirza ◽

Cornelia Volz ◽

Marcus Karlstetter ◽

Monica Langiu ◽

Aleksandra Somogyi ◽

...

Keyword(s):

Mouse Model ◽

Beneficial Effect ◽

Retinal Degeneration ◽

Neuronal Ceroid Lipofuscinosis ◽

Glial Activation ◽

Ceroid Lipofuscinosis

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Antigen presenting cell abnormalities in the Cln3 −/− mouse model of juvenile neuronal ceroid lipofuscinosis

Biochimica et Biophysica Acta (BBA) - Molecular Basis of Disease ◽

10.1016/j.bbadis.2016.04.011 ◽

2016 ◽

Vol 1862 (7) ◽

pp. 1324-1336 ◽

Cited By ~ 3

Author(s):

Samantha L. Hersrud ◽

Attila D. Kovács ◽

David A. Pearce

Keyword(s):

Mouse Model ◽

Neuronal Ceroid Lipofuscinosis ◽

Antigen Presenting Cell ◽

Juvenile Neuronal Ceroid Lipofuscinosis ◽

Ceroid Lipofuscinosis ◽

Antigen Presenting

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