OBJECTIVE -1,5-anhydroglucitol (1,5-AG) is a short-term marker of metabolic control in diabetes. Its renal loss is stimulated in hyperglycemic conditions by glycosuria, which results in a lowered plasma concentration. As a low renal threshold for glucose has been described in hepatocyte nuclear factor-1␣ (HNF-1␣) maturity-onset diabetes of the young (MODY), the 1,5-AG level may be altered in these patients. The purpose of this study was to assess the 1,5-AG levels in patients with HNF-1␣ MODY and in type 2 diabetic subjects with a similar degree of metabolic control. In addition, we aimed to evaluate this particle as a biomarker for HNF-1␣ MODY.RESEARCH DESIGN AND METHODS -We included 33 diabetic patients from the Polish Nationwide Registry of MODY. In addition, we examined 43 type 2 diabetic patients and 47 nondiabetic control subjects. The 1,5-AG concentration was measured with an enzymatic assay (GlycoMark). Receiver operating characteristic (ROC) curve analysis was used to evaluate 1,5-AG as a screening marker for HNF-1␣ MODY.RESULTS -The mean 1,5-AG plasma concentration in diabetic HNF-1␣ mutation carriers was 5.9 g/ml, and it was lower than that in type 2 diabetic patients (11.0 g/ml, P ϭ 0.003) and in nondiabetic control subjects (23.9 g/ml, P Ͻ 0.00005). The ROC curve analysis revealed 85.7% sensitivity and 80.0% specificity of 1,5-AG in screening for HNF-1␣ MODY at the criterion of Ͻ6.5 g/ml in patients with an A1C level between 6.5 and 9.0%.CONCLUSIONS -1,5-AG may be a useful biomarker for differential diagnosis of patients with HNF-1␣ MODY with a specific range of A1C, although this requires further investigation. However, the clinical use of this particle in diabetic HNF-1␣ mutation carriers for metabolic control has substantial limitations.