Ontology highlight
ABSTRACT:
SUBMITTER: Wang M
PROVIDER: S-EPMC2822118 | biostudies-literature | 2010 Mar
REPOSITORIES: biostudies-literature
Wang M M Ye R R Barron E E Baumeister P P Mao C C Luo S S Fu Y Y Luo B B Dubeau L L Hinton D R DR Lee A S AS
Cell death and differentiation 20091009 3
Neurodegenerative diseases are often associated with dysfunction in protein quality control. The endoplasmic reticulum (ER), a key site for protein synthesis, senses stressful conditions by activating the unfolded protein response (UPR). In this study we report the creation of a novel mouse model in which GRP78/BiP, a major ER chaperone and master regulator of UPR, is specifically eliminated in Purkinje cells (PCs). GRP78-depleted PCs activate UPR including the induction of GRP94, PDI, CHOP and ...[more]