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今月の分子 293: CFTRと嚢胞性線維症(CFTR and Cystic Fibrosis)

嚢胞性線維症は、粘液が濃くなって気道閉塞や慢性肺感染症を引き起こしてしまう遺伝病で、イオンや水の流れを制御するCFTRの遺伝子が変異して正常に機能しなくなることが原因になっています。このCFTRの機能を補う3種類の薬剤が治療薬として用いられ、また新たな治療薬の開発につながることを期待して別のイオンチャネルの研究もおこなわれている、というお話です。

詳しくは今月の分子「293: CFTRと嚢胞性線維症(CFTR and Cystic Fibrosis)」をご覧ください。

日本語訳(PDBj)

https://numon.pdbj.org/mom/293?l=ja

Cystic fibrosis is a genetic disease in which mucus thickens, leading to airway blockage and chronic lung infections. It is caused by a fault of the CFTR, which controls the flow of ions and water, causing it to no longer function properly. Three types of drugs that enhance the function of CFTR are used as therapeutic agents, and another ion channel is also researched in the hope of finding additional ways to treat it.

For details, please refer to the Molecule of the Month article 293: CFTR and Cystic Fibrosis

Original English Article(RCSB PDB)

https://pdb101.rcsb.org/motm/293

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