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[This corrects the article DOI 10.3389/fendo.2021.727915.]. Glucokinase (GCK) plays a central role in glucose regulation. The heterozygous mutations of can cause a monogenic form of diabetes, maturity-onset diabetes of the young (MODY) directly. In our study, we aimed to explore the mechanism of the novel mutation p.Ala259Thr leading to glucokinase deficiency and hyperglycemia. Thirty early-onset diabetes pedigrees were referred to whole exome sequencing for novel mutations identification. Purified wild-type and mutant GCK pr