Sana Hasan , Mohammad Salman Khan , M. Cecilia Lansang
{"title":"囊性纤维化跨膜传导调节剂对糖耐量受损和囊性纤维化相关糖尿病的影响","authors":"Sana Hasan , Mohammad Salman Khan , M. Cecilia Lansang","doi":"10.1016/j.jcte.2022.100301","DOIUrl":null,"url":null,"abstract":"<div><p>Cystic fibrosis (CF) is an autosomal recessive disorder, with a prevalence of 1 in 2,500 live births. It is caused by mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) gene. With the significant advancement in CFTR-directed therapies, life expectancy of CF patients has steadily increased. With improved survival, CF related co-morbidities have become more apparent. The most common endocrine complication includes Cystic fibrosis related diabetes (CFRD). Impaired glucose tolerance and insulin deficiency in CFRD leads to a decline in pulmonary function in CF patients. Here we review the underlying mechanisms involved in the pathogenesis of CFRD, focusing on the role of CFTR in the regulation of insulin secretion from the β-cell. We then discuss CFTR modulators and their effect on impaired glucose tolerance and CFRD.</p></div>","PeriodicalId":46328,"journal":{"name":"Journal of Clinical and Translational Endocrinology","volume":"29 ","pages":"Article 100301"},"PeriodicalIF":4.2000,"publicationDate":"2022-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/6e/2c/main.PMC9209718.pdf","citationCount":"4","resultStr":"{\"title\":\"The effect of cystic fibrosis transmembrane conductance regulator modulators on impaired glucose tolerance and cystic fibrosis related diabetes\",\"authors\":\"Sana Hasan , Mohammad Salman Khan , M. Cecilia Lansang\",\"doi\":\"10.1016/j.jcte.2022.100301\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"<div><p>Cystic fibrosis (CF) is an autosomal recessive disorder, with a prevalence of 1 in 2,500 live births. It is caused by mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) gene. With the significant advancement in CFTR-directed therapies, life expectancy of CF patients has steadily increased. With improved survival, CF related co-morbidities have become more apparent. The most common endocrine complication includes Cystic fibrosis related diabetes (CFRD). Impaired glucose tolerance and insulin deficiency in CFRD leads to a decline in pulmonary function in CF patients. Here we review the underlying mechanisms involved in the pathogenesis of CFRD, focusing on the role of CFTR in the regulation of insulin secretion from the β-cell. We then discuss CFTR modulators and their effect on impaired glucose tolerance and CFRD.</p></div>\",\"PeriodicalId\":46328,\"journal\":{\"name\":\"Journal of Clinical and Translational Endocrinology\",\"volume\":\"29 \",\"pages\":\"Article 100301\"},\"PeriodicalIF\":4.2000,\"publicationDate\":\"2022-09-01\",\"publicationTypes\":\"Journal Article\",\"fieldsOfStudy\":null,\"isOpenAccess\":false,\"openAccessPdf\":\"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/6e/2c/main.PMC9209718.pdf\",\"citationCount\":\"4\",\"resultStr\":null,\"platform\":\"Semanticscholar\",\"paperid\":null,\"PeriodicalName\":\"Journal of Clinical and Translational Endocrinology\",\"FirstCategoryId\":\"1085\",\"ListUrlMain\":\"https://www.sciencedirect.com/science/article/pii/S2214623722000096\",\"RegionNum\":0,\"RegionCategory\":null,\"ArticlePicture\":[],\"TitleCN\":null,\"AbstractTextCN\":null,\"PMCID\":null,\"EPubDate\":\"\",\"PubModel\":\"\",\"JCR\":\"Q1\",\"JCRName\":\"ENDOCRINOLOGY & METABOLISM\",\"Score\":null,\"Total\":0}","platform":"Semanticscholar","paperid":null,"PeriodicalName":"Journal of Clinical and Translational Endocrinology","FirstCategoryId":"1085","ListUrlMain":"https://www.sciencedirect.com/science/article/pii/S2214623722000096","RegionNum":0,"RegionCategory":null,"ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"Q1","JCRName":"ENDOCRINOLOGY & METABOLISM","Score":null,"Total":0}
The effect of cystic fibrosis transmembrane conductance regulator modulators on impaired glucose tolerance and cystic fibrosis related diabetes
Cystic fibrosis (CF) is an autosomal recessive disorder, with a prevalence of 1 in 2,500 live births. It is caused by mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) gene. With the significant advancement in CFTR-directed therapies, life expectancy of CF patients has steadily increased. With improved survival, CF related co-morbidities have become more apparent. The most common endocrine complication includes Cystic fibrosis related diabetes (CFRD). Impaired glucose tolerance and insulin deficiency in CFRD leads to a decline in pulmonary function in CF patients. Here we review the underlying mechanisms involved in the pathogenesis of CFRD, focusing on the role of CFTR in the regulation of insulin secretion from the β-cell. We then discuss CFTR modulators and their effect on impaired glucose tolerance and CFRD.