Why all MODY variants are dominantly inherited: a hypothesis

Meihang Li; Jean-Baptiste Rivière; Constantin Polychronakos

doi:10.1016/j.tig.2021.10.001

Why all MODY variants are dominantly inherited: a hypothesis

Trends Genet. 2022 Apr;38(4):321-324. doi: 10.1016/j.tig.2021.10.001. Epub 2021 Oct 22.

Authors

Meihang Li¹, Jean-Baptiste Rivière², Constantin Polychronakos³

Affiliations

¹ Clinical Research Center, Maoming People's Hospital, 101 Weimin Road, Maoming 52500, Guangdong, China; The Biomedical Sciences Institute of Qingdao University (Qingdao Branch of SJTU Bio-X Institutes), Qingdao University, 308 Ningxia road, Qingdao, Shangdong Province, China; Zhejiang MaiDa Gene Tech, 68 Xinchi road, Zhoushan, Zhejiang Province, China; School of Medicine, Jinan University, 855 Xingye East Road, Panyu, Guangzhou, Guangdong Province, China. Electronic address: [email protected].
² Research Institute of McGill University Health Centre, 1001 Decarie Boulevard, Montreal, QC, Canada. Electronic address: [email protected].
³ Zhejiang MaiDa Gene Tech, 68 Xinchi road, Zhoushan, Zhejiang Province, China; Research Institute of McGill University Health Centre, 1001 Decarie Boulevard, Montreal, QC, Canada; Zhejiang University School of Medicine, 866 Yuhangtang road, Hangzhou, Zhejiang Province, China. Electronic address: [email protected].

PMID: 34696899
DOI: 10.1016/j.tig.2021.10.001

Abstract

Maturity-onset diabetes in the young (MODY) comprises monogenic phenotypes of young-onset, insulinopenic diabetes. All its forms are dominantly inherited. Why? Are the pancreatic β cells only harmed by heterozygous variants? We propose that recessive MODYs do exist but have escaped detection due to lack of family history suggestive of monogenic inheritance.

Keywords: diabetes; family history; monogenic; polygenic; recessive MODY.

Publication types

Research Support, Non-U.S. Gov't

MeSH terms

Diabetes Mellitus, Type 2* / genetics
Humans
Insulin-Secreting Cells*
Mutation
Phenotype

Supplementary concepts

Mason-Type Diabetes