Title : Role of Proinsulin Self-Association in Mutant INS Gene-Induced Diabetes of Youth.

Pub. Date : 2020 May

PMID : 32139596






1 Functional Relationships(s)
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1 Specifically, we find that proinsulin Tyr-B16, which is a key residue in normal proinsulin dimerization, helps confer dominant-negative behavior of MIDY mutant proinsulin-C(A7)Y. Substitutions of Tyr-B16 with ether Ala, Asp, or Pro in proinsulin-C(A7)Y each decrease the abnormal interactions between the MIDY mutant and proinsulin-WT, rescuing proinsulin-WT export, limiting ER stress, and increasing insulin production in beta-cells and human islets. Aspartic Acid insulin Homo sapiens