scholarly journals pH-dependent localization of Btn1p in the yeast model for Batten disease

2010 ◽  
Vol 4 (1) ◽  
pp. 120-125 ◽  
Author(s):  
D. M. Wolfe ◽  
S. Padilla-Lopez ◽  
S. P. Vitiello ◽  
D. A. Pearce
Keyword(s):  
Batten Disease ◽  
Ph Dependent ◽  
Yeast Model

scholarly journals A yeast model for the study of Batten disease

1998 ◽  
Vol 95 (12) ◽  
pp. 6915-6918 ◽  
Author(s):  
D. A. Pearce ◽  
F. Sherman
Keyword(s):  
Batten Disease ◽  
Yeast Model

scholarly journals Absence of Btn1p in the yeast model for juvenile Batten disease may cause arginine to become toxic to yeast cells

2007 ◽  
Vol 16 (9) ◽  
pp. 1007-1016 ◽  
Author(s):  
Seasson Phillips Vitiello ◽  
Devin M. Wolfe ◽  
David A. Pearce
Keyword(s):  
Batten Disease ◽  
Yeast Cells ◽  
Juvenile Batten Disease ◽  
Yeast Model

scholarly journals Nitric Oxide Signaling Is Disrupted in the Yeast Model for Batten Disease

2007 ◽  
Vol 18 (7) ◽  
pp. 2755-2767 ◽  
Author(s):  
Nuno S. Osório ◽  
Agostinho Carvalho ◽  
Agostinho J. Almeida ◽  
Sérgio Padilla-Lopez ◽  
Cecília Leão ◽  
...  
Keyword(s):  
Oxidative Stress ◽  
Nitric Oxide ◽  
Batten Disease ◽  
Cell Types ◽  
Neuronal Ceroid Lipofuscinoses ◽  
Juvenile Form ◽  
Nitric Oxide Signaling ◽  
Induced Apoptosis ◽  
And Oxidative Stress ◽  
Yeast Model

The juvenile form of neuronal ceroid lipofuscinoses (JNCLs), or Batten disease, results from mutations in the CLN3 gene, and it is characterized by the accumulation of lipopigments in the lysosomes of several cell types and by extensive neuronal death. We report that the yeast model for JNCL (btn1-Δ) that lacks BTN1, the homologue to human CLN3, has increased resistance to menadione-generated oxidative stress. Expression of human CLN3 complemented the btn1-Δ phenotype, and equivalent Btn1p/Cln3 mutations correlated with JNCL severity. We show that the previously reported decreased levels of l-arginine in btn1-Δ limit the synthesis of nitric oxide (·NO) in both physiological and oxidative stress conditions. This defect in ·NO synthesis seems to suppress the signaling required for yeast menadione-induced apoptosis, thus explaining btn1-Δ phenotype of increased resistance. We propose that in JNCL, a limited capacity to synthesize ·NO directly caused by the absence of Cln3 function may contribute to the pathology of the disease.

scholarly journals Phenotypic reversal of the btn1 defects in yeast by chloroquine: A yeast model for Batten disease

1999 ◽  
Vol 96 (20) ◽  
pp. 11341-11345 ◽  
Author(s):  
D. A. Pearce ◽  
C. J. Carr ◽  
B. Das ◽  
F. Sherman
Keyword(s):  
Batten Disease ◽  
Phenotypic Reversal ◽  
Yeast Model

Deletion of btn1, an orthologue of CLN3, increases glycolysis and perturbs amino acid metabolism in the fission yeast model of Batten disease

2010 ◽  
Vol 6 (6) ◽  
pp. 1093 ◽  
Author(s):  
Michael R. Pears ◽  
Sandra Codlin ◽  
Rebecca L. Haines ◽  
Ian J. White ◽  
Russell J. Mortishire-Smith ◽  
...  
Keyword(s):  
Amino Acid ◽  
Fission Yeast ◽  
Amino Acid Metabolism ◽  
Acid Metabolism ◽  
Batten Disease ◽  
Yeast Model
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