These tools will no longer be maintained as of December 31, 2024. Archived website can be found here. PubMed4Hh GitHub repository can be found here. Contact NLM Customer Service if you have questions.
285 related articles for article (PubMed ID: 30444886)
1. Capitalizing on the heterogeneous effects of CFTR nonsense and frameshift variants to inform therapeutic strategy for cystic fibrosis. Sharma N; Evans TA; Pellicore MJ; Davis E; Aksit MA; McCague AF; Joynt AT; Lu Z; Han ST; Anzmann AF; Lam AN; Thaxton A; West N; Merlo C; Gottschalk LB; Raraigh KS; Sosnay PR; Cotton CU; Cutting GR PLoS Genet; 2018 Nov; 14(11):e1007723. PubMed ID: 30444886 [TBL] [Abstract][Full Text] [Related]
2. Integrity and Stability of PTC Bearing CFTR mRNA and Relevance to Future Modulator Therapies in Cystic Fibrosis. Clarke LA; Luz VCC; Targowski S; Ramalho SS; Farinha CM; Amaral MD Genes (Basel); 2021 Nov; 12(11):. PubMed ID: 34828417 [TBL] [Abstract][Full Text] [Related]
3. 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. Clarke LA; Awatade NT; Felício VM; Silva IA; Calucho M; Pereira L; Azevedo P; Cavaco J; Barreto C; Bertuzzo C; Gartner S; Beekman J; Amaral MD Hum Mutat; 2019 Mar; 40(3):326-334. PubMed ID: 30488522 [TBL] [Abstract][Full Text] [Related]
4. Exon-skipping antisense oligonucleotides for cystic fibrosis therapy. Kim YJ; Sivetz N; Layne J; Voss DM; Yang L; Zhang Q; Krainer AR Proc Natl Acad Sci U S A; 2022 Jan; 119(3):. PubMed ID: 35017301 [TBL] [Abstract][Full Text] [Related]
5. Downstream Alternate Start Site Allows N-Terminal Nonsense Variants to Escape NMD and Results in Functional Recovery by Readthrough and Modulator Combination. Bowling A; Eastman A; Merlo C; Lin G; West N; Patel S; Cutting G; Sharma N J Pers Med; 2022 Sep; 12(9):. PubMed ID: 36143233 [TBL] [Abstract][Full Text] [Related]
6. Functional Restoration of CFTR Nonsense Mutations in Intestinal Organoids. de Poel E; Spelier S; Suen SWF; Kruisselbrink E; Graeber SY; Mall MA; Weersink EJM; van der Eerden MM; Koppelman GH; van der Ent CK; Beekman JM J Cyst Fibros; 2022 Mar; 21(2):246-253. PubMed ID: 34666947 [TBL] [Abstract][Full Text] [Related]
7. 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 Hum Mol Genet; 2017 Aug; 26(16):3116-3129. PubMed ID: 28575328 [TBL] [Abstract][Full Text] [Related]
8. Functional rescue of c.3846G>A (W1282X) in patient-derived nasal cultures achieved by inhibition of nonsense mediated decay and protein modulators with complementary mechanisms of action. Laselva O; Eckford PD; Bartlett C; Ouyang H; Gunawardena TN; Gonska T; Moraes TJ; Bear CE J Cyst Fibros; 2020 Sep; 19(5):717-727. PubMed ID: 31831337 [TBL] [Abstract][Full Text] [Related]
9. Gene-specific nonsense-mediated mRNA decay targeting for cystic fibrosis therapy. Kim YJ; Nomakuchi T; Papaleonidopoulou F; Yang L; Zhang Q; Krainer AR Nat Commun; 2022 May; 13(1):2978. PubMed ID: 35624092 [TBL] [Abstract][Full Text] [Related]
10. Health Characteristics of Patients with Cystic Fibrosis whose Genotype Includes a Variant of the Nucleotide Sequence c.3140-16T>A and Functional Analysis of this Variant. Kondratyeva E; Bukharova T; Efremova A; Melyanovskaya Y; Bulatenko N; Davydenko K; Filatova A; Skoblov M; Krasovsky S; Petrova N; Polyakov A; Adyan T; Amelina E; Shadrina V; Zhekaite E; Zodbinova A; Chernyak A; Zinchenko R; Kutsev S; Goldshtein D Genes (Basel); 2021 May; 12(6):. PubMed ID: 34071719 [TBL] [Abstract][Full Text] [Related]
11. Small-molecule eRF3a degraders rescue CFTR nonsense mutations by promoting premature termination codon readthrough. Lee RE; Lewis CA; He L; Bulik-Sullivan EC; Gallant SC; Mascenik TM; Dang H; Cholon DM; Gentzsch M; Morton LC; Minges JT; Theile JW; Castle NA; Knowles MR; Kimple AJ; Randell SH J Clin Invest; 2022 Sep; 132(18):. PubMed ID: 35900863 [TBL] [Abstract][Full Text] [Related]
12. Caution advised in the use of CFTR modulator treatment for individuals harboring specific CFTR variants. Raraigh KS; Lewis MH; Collaco JM; Corey M; Penland CM; Stephenson AL; Rommens JM; Castellani C; Cutting GR J Cyst Fibros; 2022 Sep; 21(5):856-860. PubMed ID: 35527187 [TBL] [Abstract][Full Text] [Related]
13. Nonsense-mediated RNA Decay Pathway Inhibition Restores Expression and Function of W1282X CFTR. Keenan MM; Huang L; Jordan NJ; Wong E; Cheng Y; Valley HC; Mahiou J; Liang F; Bihler H; Mense M; Guo S; Monia BP Am J Respir Cell Mol Biol; 2019 Sep; 61(3):290-300. PubMed ID: 30836009 [TBL] [Abstract][Full Text] [Related]
14. Open reading frame correction using splice-switching antisense oligonucleotides for the treatment of cystic fibrosis. Michaels WE; Pena-Rasgado C; Kotaria R; Bridges RJ; Hastings ML Proc Natl Acad Sci U S A; 2022 Jan; 119(3):. PubMed ID: 35017302 [No Abstract] [Full Text] [Related]
15. CFTR mRNAs with nonsense codons are degraded by the SMG6-mediated endonucleolytic decay pathway. Sanderlin EJ; Keenan MM; Mense M; Revenko AS; Monia BP; Guo S; Huang L Nat Commun; 2022 Apr; 13(1):2344. PubMed ID: 35487895 [TBL] [Abstract][Full Text] [Related]
16. Synthetic aminoglycosides efficiently suppress cystic fibrosis transmembrane conductance regulator nonsense mutations and are enhanced by ivacaftor. Xue X; Mutyam V; Tang L; Biswas S; Du M; Jackson LA; Dai Y; Belakhov V; Shalev M; Chen F; Schacht J; J Bridges R; Baasov T; Hong J; Bedwell DM; Rowe SM Am J Respir Cell Mol Biol; 2014 Apr; 50(4):805-16. PubMed ID: 24251786 [TBL] [Abstract][Full Text] [Related]
17. Use of adenine base editing and homology-independent targeted integration strategies to correct the cystic fibrosis causing variant, W1282X. Mention K; Cavusoglu-Doran K; Joynt AT; Santos L; Sanz D; Eastman AC; Merlo C; Langfelder-Schwind E; Scallan MF; Farinha CM; Cutting GR; Sharma N; Harrison PT Hum Mol Genet; 2023 Nov; 32(23):3237-3248. PubMed ID: 37649273 [TBL] [Abstract][Full Text] [Related]
18. The CFTR frameshift mutation 3905insT and its effect at transcript and protein level. Sanz J; von Känel T; Schneider M; Steiner B; Schaller A; Gallati S Eur J Hum Genet; 2010 Feb; 18(2):212-7. PubMed ID: 19724303 [TBL] [Abstract][Full Text] [Related]