scholarly journals 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

2014 ◽  
Vol 25 (3) ◽  
pp. 223-239 ◽  
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

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

Epilepsia ◽  
2002 ◽  
Vol 43 (10) ◽  
pp. 1137-1140 ◽  
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

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

2004 ◽  
Vol 1023 (2) ◽  
pp. 231-242 ◽  
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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