CFTR potentiators partially restore channel function to A561E-CFTR, a cystic fibrosis mutant with a similar mechanism of dysfunction as F508del-CFTR
about
Impact of the F508del mutation on ovine CFTR, a Cl- channel with enhanced conductance and ATP-dependent gating.Cystic fibrosis transmembrane conductance regulator (CFTR) potentiators protect G551D but not ΔF508 CFTR from thermal instability.Measurements of Functional Responses in Human Primary Lung Cells as a Basis for Personalized Therapy for Cystic Fibrosis.From CFTR biology toward combinatorial pharmacotherapy: expanded classification of cystic fibrosis mutations.Development of Automated Patch Clamp Technique to Investigate CFTR Chloride Channel Function.Two Small Molecules Restore Stability to a Subpopulation of the Cystic Fibrosis Transmembrane Conductance Regulator with the Predominant Disease-causing Mutation.Chaperones rescue the energetic landscape of mutant CFTR at single molecule and in cell.Partial rescue of F508del-CFTR channel gating with modest improvement of protein processing, but not stability by a dual-acting small molecule.Peripheral Protein Quality Control as a Novel Drug Target for CFTR Stabilizer
P2860
Q30372632-4FC3EEEC-D579-498D-BC66-37C685312489Q34155612-2BE26456-06F4-4D57-8555-F7CA4667133DQ35681573-B1B9FD92-F2D5-4742-BAEE-CEDE22327604Q36574664-9B96E19A-3670-47B8-B2F1-97F46F4B3CE7Q37740542-B6EBC4EA-03CE-4FD2-8E7B-4130A3C16DB2Q38721354-4519A8AC-D14A-4FDF-937A-EFF4993F054EQ41565850-5632D081-A19A-4139-AC6F-E63D19B535ABQ47214928-0FD6D1F6-78C4-42F2-96A9-B398B765E7C5Q57490973-B8B837A6-BF8C-4716-AAAF-41EEFFE972D7
P2860
CFTR potentiators partially restore channel function to A561E-CFTR, a cystic fibrosis mutant with a similar mechanism of dysfunction as F508del-CFTR
description
2014 nî lūn-bûn
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name
CFTR potentiators partially re ...... of dysfunction as F508del-CFTR
@en
type
label
CFTR potentiators partially re ...... of dysfunction as F508del-CFTR
@en
prefLabel
CFTR potentiators partially re ...... of dysfunction as F508del-CFTR
@en
P2093
P2860
P356
P1476
CFTR potentiators partially re ...... of dysfunction as F508del-CFTR
@en
P2093
Avgi Loizidou
Bethan R Hawley
David N Sheppard
Luc A Bugeja
Ross Warner
Yiting Wang
Zhiwei Cai
P2860
P304
P356
10.1111/BPH.12791
P407
P577
2014-09-05T00:00:00Z