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

IF 4.2 Q1 ENDOCRINOLOGY & METABOLISM
Sana Hasan , Mohammad Salman Khan , M. Cecilia Lansang
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引用次数: 4

摘要

囊性纤维化(CF)是一种常染色体隐性遗传病,患病率为1 / 2,500活产。它是由囊性纤维化跨膜传导调节基因(CFTR)突变引起的。随着cftr导向治疗的显著进步,CF患者的预期寿命稳步提高。随着生存率的提高,CF相关的合并症变得更加明显。最常见的内分泌并发症包括囊性纤维化相关性糖尿病。CFRD患者糖耐量受损和胰岛素缺乏导致CF患者肺功能下降。本文综述了CFRD的发病机制,重点讨论了CFTR在调节β细胞分泌胰岛素中的作用。然后我们讨论CFTR调节剂及其对糖耐量受损和CFRD的影响。
本文章由计算机程序翻译,如有差异,请以英文原文为准。

The effect of cystic fibrosis transmembrane conductance regulator modulators on impaired glucose tolerance and cystic fibrosis related diabetes

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.

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来源期刊
CiteScore
6.10
自引率
0.00%
发文量
24
审稿时长
16 weeks
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