Coordinated expression and assembly of BiP, p58<sup>IPK</sup>, and ER chaperone complexes maximize proinsulin folding in pancreatic β cells.

Jang, Insook; Duffey, Alec; Itkin-Ansari, Pamela; Arvan, Peter; Kaufman, Randal J · Proc Natl Acad Sci U S A · 2026

basic_science · Level V

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Abstract

Proper proinsulin folding in the endoplasmic reticulum (ER) is prerequisite to producing bioactive insulin, and proinsulin misfolding causing β cell ER stress accompanies pancreatic β cell dysfunction in type 2 diabetes (T2D). How (and which) ER chaperones coordinate to prevent proinsulin misfolding is largely unknown other than an unspecified dependence on the hsp70 member, BiP. A genetically engineered mouse enables efficient, specific pulldown of endogenous islet β cell BiP (GRP78, the major HSP70 ER chaperone) in complexes with client proteins. We demonstrate that BiP assembles in various protein complexes (including cochaperones p58<sup>IPK</sup>, GRP170, ERdj3, and oxidoreductases PDIA1 and PDIA6) that specifically bind to nonnative proinsulin. BiP requires p58<sup>IPK</sup> for productive proinsulin folding, whereas nonstoichiometric BiP excess actually hinders proinsulin folding. Coordinated and dyscoordinated BiP/cochaperone assembly in response to demand for proinsulin highlights physiologic and pathophysiologic conditions, respectively, offering a potential check point for therapeutic intervention.