In the unfolded protein response (UPR) signaling pathway, accumulation of
unfolded proteins in the endoplasmic reticulum (ER) activates a transmembrane
kinase/ribonuclease Ire1, which causes the transcriptional induction of
ER-resident chaperones, including BiP/Kar2. It was previously hypothesized
that BiP/Kar2 plays a direct role in the signaling mechanism. In this model,
association of BiP/Kar2 with Ire1 represses the UPR pathway while under
conditions of ER stress, BiP/Kar2 dissociation leads to activation. To test
this model, we analyzed five temperature-sensitive alleles of the yeast
KAR2 gene. When cells carrying a mutation in the Kar2
substrate-binding domain were incubated at the restrictive temperature,
association of Kar2 to Ire1 was disrupted, and the UPR pathway was activated
even in the absence of extrinsic ER stress. Conversely, cells carrying a
mutation in the Kar2 ATPase domain, in which Kar2 poorly dissociated from Ire1
even in the presence of tunicamycin, a potent inducer of ER stress, were
unable to activate the pathway. Our findings provide strong evidence in
support of BiP/Kar2-dependent Ire1 regulation model and suggest that Ire1
associates with Kar2 as a chaperone substrate. We speculate that recognition
of unfolded proteins is based on their competition with Ire1 for binding with
BiP/Kar2.