Bridges RJ - Search Results

1

CFTR bearing variant p.Phe312del exhibits function inconsistent with phenotype and negligible response to ivacaftor.

Raraigh KS, Paul KC, Goralski JL, Worthington EN, Faino AV, Sciortino S, Wang Y, Aksit MA, Ling H, Osorio DL, Onchiri FM, Patel SU, Merlo CA, Montemayor K, Gibson RL, West NE, Thakerar A, Bridges RJ, Sheppard DN, Sharma N, Cutting GR. Raraigh KS, et al. Among authors: bridges rj. JCI Insight. 2022 Mar 22;7(6):e148841. doi: 10.1172/jci.insight.148841. JCI Insight. 2022. PMID: 35315358 Free PMC article.

2

CFTR modulator theratyping: Current status, gaps and future directions.

Clancy JP, Cotton CU, Donaldson SH, Solomon GM, VanDevanter DR, Boyle MP, Gentzsch M, Nick JA, Illek B, Wallenburg JC, Sorscher EJ, Amaral MD, Beekman JM, Naren AP, Bridges RJ, Thomas PJ, Cutting G, Rowe S, Durmowicz AG, Mense M, Boeck KD, Skach W, Penland C, Joseloff E, Bihler H, Mahoney J, Borowitz D, Tuggle KL. Clancy JP, et al. Among authors: bridges rj. J Cyst Fibros. 2019 Jan;18(1):22-34. doi: 10.1016/j.jcf.2018.05.004. Epub 2018 Jun 20. J Cyst Fibros. 2019. PMID: 29934203 Free PMC article. Review.

3

Identification of the amino acids inserted during suppression of CFTR nonsense mutations and determination of their functional consequences.

Xue X, Mutyam V, Thakerar A, Mobley J, Bridges RJ, Rowe SM, Keeling KM, Bedwell DM. Xue X, et al. Among authors: bridges rj. Hum Mol Genet. 2017 Aug 15;26(16):3116-3129. doi: 10.1093/hmg/ddx196. Hum Mol Genet. 2017. PMID: 28575328 Free PMC article.

4

Antisense oligonucleotide-mediated correction of CFTR splicing improves chloride secretion in cystic fibrosis patient-derived bronchial epithelial cells.

Michaels WE, Bridges RJ, Hastings ML. Michaels WE, et al. Among authors: bridges rj. Nucleic Acids Res. 2020 Jul 27;48(13):7454-7467. doi: 10.1093/nar/gkaa490. Nucleic Acids Res. 2020. PMID: 32520327 Free PMC article.

5

KCa3.1 potentiation stimulates Cl^- secretion in F508del and G551D CFTR-corrected primary human bronchial epithelial cells.

Devor DC, Green MD, Bridges RJ. Devor DC, et al. Among authors: bridges rj. Am J Physiol Cell Physiol. 2022 Oct 1;323(4):C1215-C1230. doi: 10.1152/ajpcell.00319.2022. Epub 2022 Sep 5. Am J Physiol Cell Physiol. 2022. PMID: 36062876 Free article.

6

Synonymous codon usage affects the expression of wild type and F508del CFTR.

Shah K, Cheng Y, Hahn B, Bridges R, Bradbury NA, Mueller DM. Shah K, et al. J Mol Biol. 2015 Mar 27;427(6 Pt B):1464-1479. doi: 10.1016/j.jmb.2015.02.003. Epub 2015 Feb 10. J Mol Biol. 2015. PMID: 25676312 Free PMC article.

7

Translating in vitro CFTR rescue into small molecule correctors for cystic fibrosis using the Library of Integrated Network-based Cellular Signatures drug discovery platform.

Strub MD, Ramachandran S, Boudko DY, Meleshkevitch EA, Pezzulo AA, Subramanian A, Liberzon A, Bridges RJ, McCray PB Jr. Strub MD, et al. Among authors: bridges rj. CPT Pharmacometrics Syst Pharmacol. 2022 Feb;11(2):240-251. doi: 10.1002/psp4.12751. Epub 2021 Dec 23. CPT Pharmacometrics Syst Pharmacol. 2022. PMID: 34877817 Free PMC article.

8

Restoration of CFTR Activity in Ducts Rescues Acinar Cell Function and Reduces Inflammation in Pancreatic and Salivary Glands of Mice.

Zeng M, Szymczak M, Ahuja M, Zheng C, Yin H, Swaim W, Chiorini JA, Bridges RJ, Muallem S. Zeng M, et al. Among authors: bridges rj. Gastroenterology. 2017 Oct;153(4):1148-1159. doi: 10.1053/j.gastro.2017.06.011. Epub 2017 Jun 19. Gastroenterology. 2017. PMID: 28634110 Free PMC article.

9

Sphingomyelinase decreases transepithelial anion secretion in airway epithelial cells in part by inhibiting CFTR-mediated apical conductance.

Cottrill KA, Peterson RJ, Lewallen CF, Koval M, Bridges RJ, McCarty NA. Cottrill KA, et al. Among authors: bridges rj. Physiol Rep. 2021 Aug;9(15):e14928. doi: 10.14814/phy2.14928. Physiol Rep. 2021. PMID: 34382377 Free PMC article.

10

Mechanistic analysis and significance of sphingomyelinase-mediated decreases in transepithelial CFTR currents in nHBEs.

Cottrill KA, Giacalone VD, Margaroli C, Bridges RJ, Koval M, Tirouvanziam R, McCarty NA. Cottrill KA, et al. Among authors: bridges rj. Physiol Rep. 2021 Sep;9(17):e15023. doi: 10.14814/phy2.15023. Physiol Rep. 2021. PMID: 34514718 Free PMC article.

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