Correctors of ΔF508 CFTR restore global conformational maturation without thermally stabilizing the mutant protein.
He L, Kota P, Aleksandrov AA, Cui L, Jensen T, Dokholyan NV, Riordan JR.
He L, et al. Among authors: jensen t.
FASEB J. 2013 Feb;27(2):536-45. doi: 10.1096/fj.12-216119. Epub 2012 Oct 26.
FASEB J. 2013.
PMID: 23104983
Free PMC article.
Here, we showed that several of these compounds, including the investigational drug VX-809, caused a much greater increase (5- to 10-fold) in maturation at 27 than at 37C (<2-fold), and the mature product remained short-lived (T(1/2)4.5 h) and thermally unstable, even t …
Here, we showed that several of these compounds, including the investigational drug VX-809, caused a much greater increase (5- to 10-fold) i …