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Updated: Jul 9, 2025

Functional Reconstitution and Channel Activity Measurements of Purified Wildtype and Mutant CFTR Protein
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Published on: March 9, 2015

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小分子校正器通过稳定连续折叠状态来使CFTR-F508del从ERAD转移

Celeste Riepe1, Magda Wąchalska1, Kirandeep K Deol2,3,4

  • 1Department of Biology, Stanford University, Stanford, CA 94305.

Molecular biology of the cell
|November 29, 2023
PubMed
概括

囊性纤维化 (CF) 治疗向囊性纤维化跨膜导电性调节器 (CFTR) 蛋白中的F508del突变. 这项研究确定了参与CFTR降解的新蛋白质,揭示了校正剂如何稳定CFTR折叠.

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