·讲座·

陈静 韩学尧 纪立农

基金项目：国家自然科学基金资助项目(30570873、30771031);国家重点基础性研究计划(973计划)资助项目(2005CB523000);国家高技术研究发展计划(863计划)资助项目(2006AA02A409)

作者单位：100044北京大学人民医院内分泌科、北京大学糖尿病中心

通讯作者: 纪立农,E-mail:jiln@bjmu.edu.cn

【提要】 年轻的成年发病型糖尿病(MODY)是单基因突变的胰岛β细胞功能缺陷所致的特殊类型糖尿病，以早发糖尿病及胰岛素分泌缺陷为主要特点。本文对新发现的MODY 7~MODY 11基因定位、基因结构、基因功能、基因突变及其临床特征进行文献综述。

【关键词】 年轻的成年发病型糖尿病,亚型

doi:10.3969/j.issn.1006-6187.2011.07.021

The newly discovered MODY subtypes

CHEN Jing, HAN Xue-yao,JI Li-nong. Department of Endocrinology, Peking University People′s Hospital, Beijing University Diabetes Center, Beijing 100044, China

Corresponding author: JI Li-nong, E-mail:jiln@bjmu.edu.cn

【Summary】Maturity onset diabetes of the young is caused by mutations in an autosomal dominant gene,which is referred as monogenic diabetes and it is characterized by an early onset of non-insulin dependent diabetes mellitus, usually before age 25 years and by defective insulin secretion of the pancreatic β-cell. There are 11 MODY subtypes at present.We made literature review on the newly discovered MODY 7~11 to introduce the gene localization, gene structure, gene function, gene mutations and clinical features of the 5 subtypes.

【Key words】Maturity onset diabetes of the young; Subtype