Ontology highlight
ABSTRACT:
SUBMITTER: Osorio NS
PROVIDER: S-EPMC1924819 | biostudies-literature | 2007 Jul
REPOSITORIES: biostudies-literature
Osório Nuno S NS Carvalho Agostinho A Almeida Agostinho J AJ Padilla-Lopez Sérgio S Leão Cecília C Laranjinha João J Ludovico Paula P Pearce David A DA Rodrigues Fernando F
Molecular biology of the cell 20070502 7
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-Delta) that lacks BTN1, the homologue to human CLN3, has increased resistance to menadione-generated oxidative stress. Expression of human CLN3 complemented the btn1-Delta phenotype, and equivalent Btn ...[more]