Phenotypic characterization of a mouse model of juvenile neuronal ceroid lipofuscinosis

Phenotypic Characterization ◽

Imaging data on characterization of retinal autofluorescent lesions in a mouse model of juvenile neuronal ceroid lipofuscinosis (CLN3 disease)

Data in Brief ◽

10.1016/j.dib.2020.106076 ◽

2020 ◽

Vol 32 ◽

pp. 106076 ◽

Cited By ~ 1

Author(s):

Qing Jun Wang ◽

Kyung Sik Jung ◽

Kabhilan Mohan ◽

Mark E. Kleinman

Keyword(s):

Mouse Model ◽

Imaging Data ◽

Ceroid Lipofuscinosis ◽

Cln3 Disease

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 ◽

Batten Disease ◽

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 ◽

Glial Activation ◽

Low Level ◽

Juvenile neuronal ceroid-lipofuscinosis: Characterization of the dyslipoproteinaemia and demonstration of membrane phospholipid and phospholipid-dependent signal transduction abnormalities in cultured skin fibroblasts

Journal of Inherited Metabolic Disease ◽

10.1007/bf00710272 ◽

1993 ◽

Vol 16 (2) ◽

pp. 308-311 ◽

Cited By ~ 10

Author(s):

M. J. Bennett ◽

S. F. Poirier ◽

L. Chern ◽

A. R. Gayton ◽

G. P. Hosking ◽

...

Keyword(s):

Signal Transduction ◽

Skin Fibroblasts ◽

Membrane Phospholipid ◽

Ceroid Lipofuscinosis ◽

Cultured Skin ◽

Dependent Signal

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 ◽

Lysosomal Storage ◽

Adeno Associated Virus ◽

Ceroid Lipofuscinosis ◽

Virus Serotype ◽

Partial Correction

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 ◽

Author Response ◽

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 ◽

Cerebellar Granule ◽

Ceroid Lipofuscinosis ◽

Altered Sensitivity

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 ◽

Batten Disease ◽

Knockout Mouse Model ◽

Ceroid Lipofuscinosis ◽

Retinal Pathology ◽

And Function

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 ◽

Antigen Presenting Cell ◽

Ceroid Lipofuscinosis ◽

Antigen Presenting

Cerebellar defects in a mouse model of juvenile neuronal ceroid lipofuscinosis

Brain Research ◽

10.1016/j.brainres.2009.02.009 ◽

2009 ◽

Vol 1266 ◽

pp. 93-107 ◽

Cited By ~ 39

Author(s):

Jill M. Weimer ◽

Jared W. Benedict ◽

Amanda L. Getty ◽

Charlie C. Pontikis ◽

Ming J. Lim ◽

...

Keyword(s):

Mouse Model ◽