A disrupted homologue of the human CLN3 or juvenile neuronal ceroid lipofuscinosis gene in Saccharomyces cerevisiae: a model to study Batten disease.


Journal Article

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.

Full Text

Duke Authors

Cited Authors

  • Guo, WX; Mao, C; Obeid, LM; Boustany, RM

Published Date

  • October 1999

Published In

Volume / Issue

  • 19 / 5

Start / End Page

  • 671 - 680

PubMed ID

  • 10384264

Pubmed Central ID

  • 10384264

International Standard Serial Number (ISSN)

  • 0272-4340

Digital Object Identifier (DOI)

  • 10.1023/a:1006992704108


  • eng

Conference Location

  • United States