scholarly journals The juvenile Batten disease protein, CLN3, and its role in regulating anterograde and retrograde post-Golgi trafficking

2012 ◽  
Vol 7 (1) ◽  
pp. 79-91 ◽  
Author(s):  
Susan L Cotman ◽  
John F Staropoli
Keyword(s):  
Batten Disease ◽  
Disease Protein ◽  
Juvenile Batten Disease

scholarly journals Immunosuppression alters disease severity in juvenile Batten disease mice

2011 ◽  
Vol 230 (1-2) ◽  
pp. 169-172 ◽  
Author(s):  
Sabrina S. Seehafer ◽  
Denia Ramirez-Montealegre ◽  
Andrew MS Wong ◽  
Chun-Hung Chan ◽  
Julian Castaneda ◽  
...  
Keyword(s):  
Disease Severity ◽  
Batten Disease ◽  
Juvenile Batten Disease

Cell death pathways in juvenile Batten disease

APOPTOSIS ◽  
2005 ◽  
Vol 10 (5) ◽  
pp. 973-985 ◽  
Author(s):  
D. A. Persaud-Sawin ◽  
R-M. N. Boustany
Keyword(s):  
Cell Death ◽  
Batten Disease ◽  
Cell Death Pathways ◽  
Juvenile Batten Disease

scholarly journals A model for Batten disease protein CLN3: Functional implications from homology and mutations

FEBS Letters ◽  
1996 ◽  
Vol 399 (1-2) ◽  
pp. 75-77 ◽  
Author(s):  
Robert W. Janes ◽  
Patricia B. Munroe ◽  
Hannah M. Mitchison ◽  
R. Mark Gardiner ◽  
Sara E. Mole ◽  
...  
Keyword(s):  
Batten Disease ◽  
Functional Implications ◽  
Disease Protein

scholarly journals Juvenile Batten Disease Past and Present Diagnosis

2016 ◽  
Vol 5 (3) ◽  
Author(s):  
V Ramakrishnan ◽  
Akram Husain RS
Keyword(s):  
Batten Disease ◽  
Juvenile Batten Disease

scholarly journals Phenotypes of Juvenile Batten Disease

1999 ◽  
Vol 13 (2) ◽  
pp. 16
Author(s):  
J Gordon Millichap
Keyword(s):  
Batten Disease ◽  
Juvenile Batten Disease

scholarly journals Age-dependent therapeutic effect of memantine in a mouse model of juvenile Batten disease

2012 ◽  
Vol 63 (5) ◽  
pp. 769-775 ◽  
Author(s):  
Attila D. Kovács ◽  
Angelika Saje ◽  
Andrew Wong ◽  
Serena Ramji ◽  
Jonathan D. Cooper ◽  
...  
Keyword(s):  
Mouse Model ◽  
Therapeutic Effect ◽  
Batten Disease ◽  
Age Dependent ◽  
Juvenile Batten Disease

scholarly journals Two Motifs Target Batten Disease Protein CLN3 to Lysosomes in Transfected Nonneuronal and Neuronal Cells

2004 ◽  
Vol 15 (3) ◽  
pp. 1313-1323 ◽  
Author(s):  
Aija Kyttälä ◽  
Gudrun Ihrke ◽  
Jouni Vesa ◽  
Michael J. Schell ◽  
J. Paul Luzio
Keyword(s):  
Neurodegenerative Disorder ◽  
Neuronal Cells ◽  
Batten Disease ◽  
Large Loop ◽  
Loop Domain ◽  
Neuronal Processes ◽  
Disease Protein ◽  
Lysosomal Targeting ◽  
Optimal Efficiency ◽  
Polytopic Membrane Protein

Batten disease is a neurodegenerative disorder resulting from mutations in CLN3, a polytopic membrane protein, whose predominant intracellular destination in nonneuronal cells is the lysosome. The topology of CLN3 protein, its lysosomal targeting mechanism, and the development of Batten disease are poorly understood. We provide experimental evidence that both the N and C termini and one large loop domain of CLN3 face the cytoplasm. We have identified two lysosomal targeting motifs that mediate the sorting of CLN3 in transfected nonneuronal and neuronal cells: an unconventional motif in the long C-terminal cytosolic tail consisting of a methionine and a glycine separated by nine amino acids [M(X)9G], and a more conventional dileucine motif, located in the large cytosolic loop domain and preceded by an acidic patch. Each motif on its own was sufficient to mediate lysosomal targeting, but optimal efficiency required both. Interestingly, in primary neurons, CLN3 was prominently seen both in lysosomes in the cell body and in endosomes, containing early endosomal antigen-1 along neuronal processes. Because there are few lysosomes in axons and peripheral parts of dendrites, the presence of CLN3 in endosomes of neurons may be functionally important. Endosomal association of the protein was independent of the two lysosomal targeting motifs.

CLN3P, the Batten disease protein, localizes to membrane lipid rafts (detergent-resistant membranes)

2004 ◽  
Vol 317 (4) ◽  
pp. 988-991 ◽  
Author(s):  
Dinesh Rakheja ◽  
Srinivas B Narayan ◽  
Johanne V Pastor ◽  
Michael J Bennett
Keyword(s):  
Lipid Rafts ◽  
Batten Disease ◽  
Membrane Lipid ◽  
Disease Protein ◽  
Detergent Resistant Membranes
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