Abstract
1. In order to investigate the biological function of the human CLN3 gene that is defective in Batten disease, we created a yeast strain by PCR-targeted disruption of the yeast gene (YHC3), which is a homologue of the human CLN3 gene. 2. The phenotypic characterization revealed that the yhc3 delta mutants are more sensitive to combined heat and alkaline stress than the wild-type strains as determined by inhibition of cell proliferation. 3. This suggests that the yhc3 delta mutant is a good model to investigate the biological function of human CLN3 gene in mammalian cells and to understand the pathophysiology of juvenile Batten disease.
Publication types
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Research Support, Non-U.S. Gov't
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Research Support, U.S. Gov't, P.H.S.
MeSH terms
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Alkalosis / physiopathology
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Antineoplastic Agents / pharmacology
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Cyclins / genetics
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Enzyme Inhibitors / pharmacology
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Humans
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Hydrogen-Ion Concentration
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Membrane Glycoproteins*
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Molecular Chaperones*
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Neuronal Ceroid-Lipofuscinoses / genetics*
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Neuronal Ceroid-Lipofuscinoses / physiopathology
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Oligomycins / pharmacology
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Proteins / genetics*
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Proteins / physiology
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Saccharomyces cerevisiae / drug effects
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Saccharomyces cerevisiae / genetics*
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Saccharomyces cerevisiae Proteins / genetics
Substances
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Antineoplastic Agents
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CLN3 protein, human
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Cyclins
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Enzyme Inhibitors
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Membrane Glycoproteins
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Molecular Chaperones
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Oligomycins
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Proteins
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Saccharomyces cerevisiae Proteins
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YHC3 protein, S cerevisiae