"MODY 6"

‐load glucose rise > 5 mmol/L on oral glucose tolerance test (HNF1A or HNF4A MODY) 4. ▶ lack of marked obesity and other metabolic syndrome features (eg, acanthosis nigricans) 5. ▶ specific features such as genitourinary tract abnormalities or renal cysts, pancreatic atrophy, hyperuricaemia or gout (HNF1B MODY) 6. ▶ history of neonatal hyperglycaemia 7. ▶ maternal line inheritance

, Sue Prosser 3, Andrew Gibson 4, Katie Kelsey 2, Ruben Mujica-Mota 2, Brad Manktelow 5, Neena Modi 6, Steve Thornton 7, Martin Pitt 1,2,*1 National Institute for Health Research: Collaborations for Leadership in Applied Health Research and Care – South West Peninsula, University of Exeter Medical School, University of Exeter, Exeter, UK2 Institute of Health Research, University of Exeter Medical

are reported to be MODY type 1~14, of which MODY 2 and 3 might be the most common forms. Although MODY is currently classified as diabetes of a single gene defect, it has become clear that mutations in rare MODYs, such as MODY 5 and MODY 6, have small mutagenic effects and low penetrance. In addition, as there are differences in the clinical phenotypes caused by the same mutation even in the same family

. MODY 5 137920 hepatocyte nuclear factor 1β One of the less common forms of MODY, with some distinctive clinical features, including atrophy of the pancreas and several forms of renal disease. Defect in HNF-1 beta gene. 5%–10% cases. MODY 6 606394 neurogenic differentiation