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Maturity-onset diabetes of the young (MODY3), a monogenic form of type II diabetes mellitus, results most commonly from mutations in hepatocyte nuclear factor 1α (HNF-1α). Diabetes-associated mutation G20R perturbs the dimerization domain of HNF-1α, an intertwined four-helix bundle. In the wild-type structure G20 participates in a Schellman motif to cap an α-helix; its dihedral angles lie in the right...
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