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Background  The molecular basis of the Turkish population with suspected maturity-onset diabetes of the young (MODY) has not been identified. This is the first study to investigate the association between  HNF1A -gene single-nucleotide polymorphisms (SNPs) and having early-onset, MODY-like diabetes mellitus in the Turkish population.    Methods  All diabetic patients ( N  = 565) who presented to our clinic between 2012 and 2015 with a clinical suspicion of MODY were included in the study. Analysis of  HNF1A, HNFB, HNF4A, GCK  gene mutations was performed using real-time polymerase chain reaction sequencing. After genetic analysis, diabetics ( n  = 46) with  HNF1A, HNF1B, HNF4A, GCK  gene mutations (diagnosed as MODY) and diabetics ( n  = 30) with  HNF1B, HNF4A, GCK  gene SNPs were excluded. Patients with early-onset, MODY-like diabetes ( n  = 486) and non-diabetic controls ( n  = 263) were included. Genetic analyses for the  HNF1A  gene  p.S487 N  (rs2464196),  p.A98V  (rs1800574) and  p.I27L  (rs1169288) SNPs were performed using Sanger-based DNA sequencing among the control group.    Results   p.S487 N  and  p.A98V  was similar between the diabetics and controls in dominant and recessive models with no association (each,  p  > 0.05).  p.I27L  GT/TT carriers (GT/TT vs. GG, OR = 1.68, 95% CI: [1. 21-2.13];  p  = 0.035) and  p.I27L  TT carriers had increased risk of having MODY-like diabetes (GT/GG vs. TT, OR = 1.56, 95% CI: [1. 14-2.57];  p  = 0.048). Family inheritance of diabetes was significantly more common in patients with the p.I27L TT genotype. The  p.I27L  SNP was modestly associated with having diabetes after adjusting for body mass index and age ( β  = 1.45, 95% CI: [1. 2-4.2];  p  = 0.036).    Conclusions  The  HNF1A  gene  p.I27L  SNP was modestly associated with having early-onset, MODY-like diabetes in the Turkish population.  HNF1A  gene p.I27L SNP might contribute to age at diabetes diagnosis and family inheritance.    Electronic supplementary material  The online version of this article (10.1186/s12902-019-0375-2) contains supplementary material, which is available to authorized users.

HNF1A gene p.I27L is associated with early-onset, maturity-onset diabetes of the young-like diabetes in Turkey