Detalhe da pesquisa
1.
Dynamic regulation of airway surface liquid pH by TMEM16A and SLC26A4 in cystic fibrosis nasal epithelia with rare mutations.
Proc Natl Acad Sci U S A
; 120(47): e2307551120, 2023 Nov 21.
Artigo
Inglês
| MEDLINE | ID: mdl-37967223
2.
Use of 2,6-diaminopurine as a potent suppressor of UGA premature stop codons in cystic fibrosis.
Mol Ther
; 31(4): 970-985, 2023 04 05.
Artigo
Inglês
| MEDLINE | ID: mdl-36641622
3.
Long-term expanding human airway organoids for disease modeling.
EMBO J
; 38(4)2019 02 15.
Artigo
Inglês
| MEDLINE | ID: mdl-30643021
4.
Responsible use of organoids in precision medicine: the need for active participant involvement.
Development
; 147(7)2020 04 06.
Artigo
Inglês
| MEDLINE | ID: mdl-32253255
5.
The SLC26A9 inhibitor S9-A13 provides no evidence for a role of SLC26A9 in airway chloride secretion but suggests a contribution to regulation of ASL pH and gastric proton secretion.
FASEB J
; 36(11): e22534, 2022 11.
Artigo
Inglês
| MEDLINE | ID: mdl-36183361
6.
CFTR Function Restoration upon Elexacaftor/Tezacaftor/Ivacaftor Treatment in Patient-Derived Intestinal Organoids with Rare CFTR Genotypes.
Int J Mol Sci
; 24(19)2023 Sep 26.
Artigo
Inglês
| MEDLINE | ID: mdl-37833986
7.
Recurrent Respiratory Syncytial Virus Infection in a CD14-Deficient Patient.
J Infect Dis
; 226(2): 258-269, 2022 08 24.
Artigo
Inglês
| MEDLINE | ID: mdl-35429403
8.
Forskolin-induced organoid swelling is associated with long-term cystic fibrosis disease progression.
Eur Respir J
; 60(2)2022 08.
Artigo
Inglês
| MEDLINE | ID: mdl-35086832
9.
An open-source high-content analysis workflow for CFTR function measurements using the forskolin-induced swelling assay.
Bioinformatics
; 36(24): 5686-5694, 2021 Apr 05.
Artigo
Inglês
| MEDLINE | ID: mdl-33367496
10.
Redefining Hypo- and Hyper-Responding Phenotypes of CFTR Mutants for Understanding and Therapy.
Int J Mol Sci
; 23(23)2022 Dec 02.
Artigo
Inglês
| MEDLINE | ID: mdl-36499495
11.
Drug Repurposing for Cystic Fibrosis: Identification of Drugs That Induce CFTR-Independent Fluid Secretion in Nasal Organoids.
Int J Mol Sci
; 23(20)2022 Oct 21.
Artigo
Inglês
| MEDLINE | ID: mdl-36293514
12.
Correction of CFTR function in intestinal organoids to guide treatment of cystic fibrosis.
Eur Respir J
; 57(1)2021 01.
Artigo
Inglês
| MEDLINE | ID: mdl-32747394
13.
A new era for people with cystic fibrosis.
Eur J Pediatr
; 180(9): 2731-2739, 2021 Sep.
Artigo
Inglês
| MEDLINE | ID: mdl-34213646
14.
Sensitive Monogenic Noninvasive Prenatal Diagnosis by Targeted Haplotyping.
Am J Hum Genet
; 101(3): 326-339, 2017 Sep 07.
Artigo
Inglês
| MEDLINE | ID: mdl-28844486
15.
The effect of premature termination codon mutations on CFTR mRNA abundance in human nasal epithelium and intestinal organoids: a basis for read-through therapies in cystic fibrosis.
Hum Mutat
; 40(3): 326-334, 2019 03.
Artigo
Inglês
| MEDLINE | ID: mdl-30488522
16.
Organoid-guided synergistic treatment of minimal function CFTR mutations with CFTR modulators, roflumilast and simvastatin: a personalised approach.
Eur Respir J
; 63(1)2024 Jan.
Artigo
Inglês
| MEDLINE | ID: mdl-37857424
17.
Strong Inhibition of Cholera Toxin B Subunit by Affordable, Polymer-Based Multivalent Inhibitors.
Bioconjug Chem
; 30(3): 785-792, 2019 03 20.
Artigo
Inglês
| MEDLINE | ID: mdl-30629410
18.
A 'catch-and-release' receptor for the cholera toxin.
Faraday Discuss
; 219(0): 112-127, 2019 10 30.
Artigo
Inglês
| MEDLINE | ID: mdl-31313796
19.
Stratifying infants with cystic fibrosis for disease severity using intestinal organoid swelling as a biomarker of CFTR function.
Eur Respir J
; 52(3)2018 09.
Artigo
Inglês
| MEDLINE | ID: mdl-30166324
20.
An inducible mouse model for microvillus inclusion disease reveals a role for myosin Vb in apical and basolateral trafficking.
Proc Natl Acad Sci U S A
; 112(40): 12408-13, 2015 Oct 06.
Artigo
Inglês
| MEDLINE | ID: mdl-26392529