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Article

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

Diabetes - 1 May 2020

Sun Jinhong, Xiong Yi, Li Xin, Haataja Leena, Chen Wei, Mir Saiful A, Lv Li, Madley Rachel, Larkin Dennis, Anjum Arfah, Dhayalan Balamurugan, Rege Nischay, Wickramasinghe Nalinda P, Weiss Michael A, Itkin-Ansari Pamela, Kaufman Randal J, Ostrov David A, Arvan Peter, Liu Ming

Abstract excerpt

Abnormal interactions between misfolded mutant and wild-type (WT) proinsulin (PI) in the endoplasmic reticulum (ER) drive the molecular pathogenesis of mutant INS gene-induced diabetes of youth (MIDY). How these abnormal interactions are initiated remains unknown. Normally, PI-WT dimerizes in the ER. Here, we suggest that the normal PI-PI contact surface, involving the B-chain, contributes to dominant-negative...

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Identifiers

PMID: 32139596
DOI: 10.2337/db19-1106
PMCID: PMC7171958

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