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团结就是力量:CFTR与SLC26A6之间的相互联盟作为囊性纤维化的治疗契机

Unity Is Strength: The Mutual Alliance between CFTR and SLC26A6 as Therapeutic Opportunity in Cystic Fibrosis.

作者信息

Pariano Marilena, Antognelli Cinzia, Romani Luigina, Costantini Claudio

机构信息

Department of Medicine and Surgery, University of Perugia, 06132 Perugia, Italy.

出版信息

Pharmaceuticals (Basel). 2024 Mar 12;17(3):367. doi: 10.3390/ph17030367.

Abstract

Patients with cystic fibrosis (PwCF) have recently experienced an unprecedented breakthrough with the adoption of modulator therapy in clinical practice. This remarkable achievement has led to the reconsideration of disease management as the increased life expectancy has gradually shifted the attention over a spectrum of extra-pulmonary manifestations that become prevalent in the aging population. It comes to be that complementary approaches that target patient co-morbidities are needed for the optimal clinical management of PwCF. A strategy would be to adjuvate the cystic fibrosis transmembrane conductance regulator (CFTR) in performing its functions in the different organs in which it is expressed. Solute carrier family 26 (SLC26) members appear ideal in this context. Indeed, they not only cooperate with CFTR in the organ-dependent regulation of ion fluxes but physically interact with it to reciprocally modulate their function. In this opinion, we summarize available evidence pointing to a physical and functional interaction between CFTR and SLC26 members, with a particular focus on SLC26A6 for its wider expression and broader anion selectivity, and then discuss how restoring the physical interaction between CFTR and SLC26A6 might be beneficial in the treatment of PwCF in the era of modulator therapy.

摘要

囊性纤维化患者(PwCF)最近在临床实践中采用调节剂疗法方面取得了前所未有的突破。这一显著成就促使人们重新思考疾病管理,因为预期寿命的增加逐渐将注意力转移到了在老年人群中普遍存在的一系列肺外表现上。事实证明,针对患者合并症的补充方法对于PwCF的最佳临床管理是必要的。一种策略是辅助囊性纤维化跨膜传导调节因子(CFTR)在其表达的不同器官中发挥功能。在这种情况下,溶质载体家族26(SLC26)成员似乎是理想的选择。事实上,它们不仅在器官依赖性离子通量调节中与CFTR合作,而且与其发生物理相互作用以相互调节其功能。在本观点中,我们总结了指向CFTR与SLC26成员之间存在物理和功能相互作用的现有证据,特别关注SLC26A6,因为其表达更广泛且阴离子选择性更宽,然后讨论在调节剂疗法时代恢复CFTR与SLC26A6之间的物理相互作用如何可能对PwCF的治疗有益。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/aadf/10974424/5fe08703055d/pharmaceuticals-17-00367-g001.jpg

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