期刊
TRENDS IN ENDOCRINOLOGY AND METABOLISM
卷 28, 期 5, 页码 377-387出版社
ELSEVIER SCIENCE LONDON
DOI: 10.1016/j.tem.2017.02.003
关键词
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资金
- Wellcome Trust [089795]
- European Research Council (ERC) [332620]
- Royal Society
- Royal Society/Wolfson Merit Award
Activating mutations in one of the two subunits of the ATP-sensitive potassium (K-ATP) channel cause neonatal diabetes (ND). This may be either transient or permanent and, in approximately 20% of patients, is associated with neuro-developmental delay. In most patients, switching from insulin to oral sulfonylurea therapy improves glycemic control and ameliorates some of the neurological disabilities. Here, we review how K-ATP channel mutations lead to the varied clinical phenotype, how sulfonylureas exert their therapeutic effects, and why their efficacy varies with individual mutations.
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