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Margarida D. Amaral - Publications

Affiliations: 
Biochemistry Faculdade de Ciências da Universidade de Lisboa 
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166 high-probability publications. We are testing a new system for linking publications to authors. You can help! If you notice any inaccuracies, please sign in and mark papers as correct or incorrect matches. If you identify any major omissions or other inaccuracies in the publication list, please let us know.

Year Citation  Score
2024 Ferreira FC, Amaral MD, Bacalhau M, Lopes-Pacheco M. PTI-801 (posenacaftor) shares a common mechanism with VX-445 (elexacaftor) to rescue p.Phe508del-CFTR. European Journal of Pharmacology. 967: 176390. PMID 38336013 DOI: 10.1016/j.ejphar.2024.176390  0.313
2023 Railean V, Rodrigues CS, Ramalho SS, Silva IAL, Bartosch J, Farinha CM, Pankonien I, Amaral MD. Personalized medicine: Function of CFTR variant p.Arg334Trp is rescued by currently available CFTR modulators. Frontiers in Molecular Biosciences. 10: 1155705. PMID 37006619 DOI: 10.3389/fmolb.2023.1155705  0.333
2023 Santos L, Nascimento R, Duarte A, Railean V, Amaral MD, Harrison PT, Gama-Carvalho M, Farinha CM. Mutation-class dependent signatures outweigh disease-associated processes in cystic fibrosis cells. Cell & Bioscience. 13: 26. PMID 36759923 DOI: 10.1186/s13578-023-00975-y  0.347
2023 Bacalhau M, Ferreira FC, Silva IAL, Buarque CD, Amaral MD, Lopes-Pacheco M. Additive Potentiation of R334W-CFTR Function by Novel Small Molecules. Journal of Personalized Medicine. 13. PMID 36675763 DOI: 10.3390/jpm13010102  0.361
2022 Bacalhau M, Ferreira FC, Kmit A, Souza FR, da Silva VD, Pimentel AS, Amaral MD, Buarque CD, Lopes-Pacheco M. Identification of Novel F508del-CFTR traffic correctors among triazole derivatives. European Journal of Pharmacology. 175396. PMID 36410419 DOI: 10.1016/j.ejphar.2022.175396  0.333
2022 Amaral MD, Harrison PT. Development of novel therapeutics for all individuals with CF (the future goes on). Journal of Cystic Fibrosis : Official Journal of the European Cystic Fibrosis Society. PMID 36319570 DOI: 10.1016/j.jcf.2022.10.007  0.305
2022 Rodenburg LW, Delpiano L, Railean V, Centeio R, Pinto MC, Smits SMA, van der Windt IS, van Hugten CFJ, van Beuningen SFB, Rodenburg RNP, van der Ent CK, Amaral MD, Kunzelmann K, Gray MA, Beekman JM, et al. Drug Repurposing for Cystic Fibrosis: Identification of Drugs That Induce CFTR-Independent Fluid Secretion in Nasal Organoids. International Journal of Molecular Sciences. 23. PMID 36293514 DOI: 10.3390/ijms232012657  0.314
2022 Amaral MD. Using the genome to correct the ion transport defect in cystic fibrosis. The Journal of Physiology. PMID 36068724 DOI: 10.1113/JP282308  0.341
2022 Ferreira JF, Silva IAL, Botelho HM, Amaral MD, Farinha CM. Absence of EPAC1 Signaling to Stabilize CFTR in Intestinal Organoids. Cells. 11. PMID 35892592 DOI: 10.3390/cells11152295  0.377
2022 Quaresma MC, Botelho HM, Pankonien I, Rodrigues CS, Pinto MC, Costa PR, Duarte A, Amaral MD. Exploring YAP1-centered networks linking dysfunctional CFTR to epithelial-mesenchymal transition. Life Science Alliance. 5. PMID 35500936 DOI: 10.26508/lsa.202101326  0.382
2022 Pankonien I, Quaresma MC, Rodrigues CS, Amaral MD. CFTR, Cell Junctions and the Cytoskeleton. International Journal of Molecular Sciences. 23. PMID 35269829 DOI: 10.3390/ijms23052688  0.364
2022 Lim SH, Snider J, Birimberg-Schwartz L, Ip W, Serralha JC, Botelho HM, Lopes-Pacheco M, Pinto MC, Moutaoufik MT, Zilocchi M, Laselva O, Esmaeili M, Kotlyar M, Lyakisheva A, Tang P, ... ... Amaral MD, et al. CFTR interactome mapping using the mammalian membrane two-hybrid high-throughput screening system. Molecular Systems Biology. 18: e10629. PMID 35156780 DOI: 10.15252/msb.202110629  0.417
2021 Ramalho SS, Silva IAL, Amaral MD, Farinha CM. Rare Trafficking CFTR Mutations Involve Distinct Cellular Retention Machineries and Require Different Rescuing Strategies. International Journal of Molecular Sciences. 23. PMID 35008443 DOI: 10.3390/ijms23010024  0.334
2021 Pinto MC, Quaresma MC, Silva IAL, Railean V, Ramalho SS, Amaral MD. Synergy in Cystic Fibrosis Therapies: Targeting SLC26A9. International Journal of Molecular Sciences. 22. PMID 34884866 DOI: 10.3390/ijms222313064  0.39
2021 Pereira C, Mazein A, Farinha CM, Gray MA, Kunzelmann K, Ostaszewski M, Balaur I, Amaral MD, Falcao AO. CyFi-MAP: an interactive pathway-based resource for cystic fibrosis. Scientific Reports. 11: 22223. PMID 34782688 DOI: 10.1038/s41598-021-01618-3  0.371
2021 Pinto MC, Silva IAL, Figueira MF, Amaral MD, Lopes-Pacheco M. Pharmacological Modulation of Ion Channels for the Treatment of Cystic Fibrosis. Journal of Experimental Pharmacology. 13: 693-723. PMID 34326672 DOI: 10.2147/JEP.S255377  0.363
2021 Roda J, Teixeira T, Ai Silva I, Silva TR, Ferreira R, Amaral MD, Oliveira G. Pediatric population with cystic fibrosis in the centre of Portugal: Candidates for new therapies. Jornal De Pediatria. PMID 34252371 DOI: 10.1016/j.jped.2021.05.010  0.384
2021 Bene Z, Fejes Z, Szanto TG, Fenyvesi F, Váradi J, Clarke LA, Panyi G, Macek M, Amaral MD, Balogh I, Nagy B. Enhanced Expression of Human Epididymis Protein 4 (HE4) Reflecting Pro-Inflammatory Status Is Regulated by CFTR in Cystic Fibrosis Bronchial Epithelial Cells. Frontiers in Pharmacology. 12: 592184. PMID 34054511 DOI: 10.3389/fphar.2021.592184  0.333
2020 Vonk AM, van Mourik P, Ramalho AS, Silva IAL, Statia M, Kruisselbrink E, Suen SWF, Dekkers JF, Vleggaar FP, Houwen RHJ, Mullenders J, Boj SF, Vries R, Amaral MD, de Boeck K, et al. Protocol for Application, Standardization and Validation of the Forskolin-Induced Swelling Assay in Cystic Fibrosis Human Colon Organoids. Star Protocols. 1: 100019. PMID 33111074 DOI: 10.1016/j.xpro.2020.100019  0.313
2020 Quaresma MC, Pankonien I, Clarke LA, Sousa LS, Silva IAL, Railean V, Doušová T, Fuxe J, Amaral MD. Mutant CFTR Drives TWIST1 mediated epithelial-mesenchymal transition. Cell Death & Disease. 11: 920. PMID 33106471 DOI: 10.1038/s41419-020-03119-z  0.404
2020 Sousa L, Pankonien I, Simões FB, Chanson M, Amaral MD. Impact of KLF4 on Cell Proliferation and Epithelial Differentiation in the Context of Cystic Fibrosis. International Journal of Molecular Sciences. 21. PMID 32937756 DOI: 10.3390/Ijms21186717  0.412
2020 da Silva Filho LVRF, Maróstica PJC, Athanazio RA, Reis FJC, Damaceno N, Paes AT, Hira AY, Schlesinger D, Kok F, Amaral MD. Extensive CFTR sequencing through NGS in Brazilian individuals with cystic fibrosis: unravelling regional discrepancies in the country. Journal of Cystic Fibrosis : Official Journal of the European Cystic Fibrosis Society. PMID 32819855 DOI: 10.1016/J.Jcf.2020.08.007  0.338
2020 Silva IAL, Doušová T, Ramalho S, Centeio R, Clarke LA, Railean V, Botelho HM, Holubová A, Valášková I, Yeh JT, Hwang TC, Farinha CM, Kunzelmann K, Amaral MD. Organoids as a personalized medicine tool for ultra-rare mutations in cystic fibrosis: The case of S955P and 1717-2A > G. Biochimica Et Biophysica Acta. Molecular Basis of Disease. 165905. PMID 32730979 DOI: 10.1016/J.Bbadis.2020.165905  0.329
2020 Sousa L, Pankonien I, Clarke LA, Silva I, Kunzelmann K, Amaral MD. KLF4 Acts as a wt-CFTR Suppressor through an AKT-Mediated Pathway. Cells. 9. PMID 32630830 DOI: 10.3390/Cells9071607  0.502
2020 Uliyakina I, Botelho HM, da Paula AC, Afonso S, Lobo MJ, Felício V, Farinha CM, Amaral MD. Full Rescue of F508del-CFTR Processing and Function by CFTR Modulators Can Be Achieved by Removal of Two Regulatory Regions. International Journal of Molecular Sciences. 21. PMID 32630527 DOI: 10.3390/Ijms21124524  0.496
2020 Lopes-Pacheco M, Silva IAL, Turner MJ, Carlile GW, Sondo E, Thomas DY, Pedemonte N, Hanrahan JW, Amaral MD. Characterization of the Mechanism of Action of RDR01752, a Novel Corrector of F508del-CFTR. Biochemical Pharmacology. 114133. PMID 32628927 DOI: 10.1016/J.Bcp.2020.114133  0.469
2020 Santos JD, Pinto FR, Ferreira JF, Amaral MD, Zaccolo M, Farinha CM. Cytoskeleton regulators CAPZA2 and INF2 associate with CFTR to control its plasma membrane levels under EPAC1 activation. The Biochemical Journal. PMID 32573649 DOI: 10.1042/Bcj20200287  0.726
2020 Bene Z, Fejes Z, Macek M, Amaral MD, Balogh I, Nagy B. Laboratory biomarkers for lung disease severity and progression in cystic fibrosis. Clinica Chimica Acta; International Journal of Clinical Chemistry. 508: 277-286. PMID 32428503 DOI: 10.1016/J.Cca.2020.05.015  0.405
2020 Pinto MC, Schreiber R, Lerias J, Ousingsawat J, Duarte A, Amaral M, Kunzelmann K. Regulation of TMEM16A by CK2 and Its Role in Cellular Proliferation. Cells. 9. PMID 32380794 DOI: 10.3390/Cells9051138  0.379
2020 Amaral MD, Quaresma MC, Pankonien I. What Role Does CFTR Play in Development, Differentiation, Regeneration and Cancer? International Journal of Molecular Sciences. 21. PMID 32365523 DOI: 10.3390/Ijms21093133  0.422
2020 Almeida C, Amaral MD. A central role of the endoplasmic reticulum in the cell emerges from its functional contact sites with multiple organelles. Cellular and Molecular Life Sciences : Cmls. 77: 4729-4745. PMID 32313974 DOI: 10.1007/S00018-020-03523-W  0.305
2020 Amaral MD, Hutt DM, Tomati V, Botelho HM, Pedemonte N. CFTR processing, trafficking and interactions. Journal of Cystic Fibrosis : Official Journal of the European Cystic Fibrosis Society. 19: S33-S36. PMID 31680043 DOI: 10.1016/J.Jcf.2019.10.017  0.503
2020 Amaral MD, Beekman JM. Activating alternative chloride channels to treat CF: Friends or Foes?: Report on the Meeting of the Basic Science Working Group in Dubrovnik, Croatia. Journal of Cystic Fibrosis : Official Journal of the European Cystic Fibrosis Society. 19: 11-15. PMID 31676346 DOI: 10.1016/J.Jcf.2019.10.005  0.387
2020 Fejes Z, Balla A, Mezei ZA, Bene Z, Macek M, Amaral MD, Balogh I, Nagy B. WS12.5 Plasma human epididymis protein 4 (HE4) levels correlate with the improvement of FEV1 in cystic fibrosis patients treated with lumacaftor/ivacaftor Journal of Cystic Fibrosis. 19. DOI: 10.1016/S1569-1993(20)30231-9  0.346
2019 Simões FB, Quaresma MC, Clarke LA, Silva IA, Pankonien I, Railean V, Kmit A, Amaral MD. TMEM16A chloride channel does not drive mucus production. Life Science Alliance. 2. PMID 31732694 DOI: 10.26508/lsa.201900462  0.322
2019 Amaral MD, Boeck Kd, Amaral M, Davies JC, Drevinek P, Elborn S, Kerem E, Lee T. Theranostics by testing CFTR modulators in patient-derived materials: The current status and a proposal for subjects with rare CFTR mutations. Journal of Cystic Fibrosis. 18: 685-692. PMID 31326274 DOI: 10.1016/J.Jcf.2019.06.010  0.351
2019 Matos AM, Pinto FR, Barros P, Amaral MD, Pepperkok R, Matos P. Inhibition of calpain 1 restores plasma membrane stability to pharmacologically rescued Phe508del-CFTR variant. The Journal of Biological Chemistry. 294: 13396-13410. PMID 31324722 DOI: 10.1074/Jbc.Ra119.008738  0.735
2019 Davies JC, Drevinek P, Elborn JS, Kerem E, Lee T, Amaral MD, de Boeck K, Davies JC, Drevinek P, Elborn JS, Kerem E, Lee T. Speeding up access to new drugs for CF: Considerations for clinical trial design and delivery. Journal of Cystic Fibrosis : Official Journal of the European Cystic Fibrosis Society. PMID 31303382 DOI: 10.1016/J.Jcf.2019.06.011  0.327
2019 Santos JD, Canato S, Carvalho AS, Botelho HM, Aloria K, Amaral MD, Matthiesen R, Falcao AO, Farinha CM. Folding Status Is Determinant over Traffic-Competence in Defining CFTR Interactors in the Endoplasmic Reticulum. Cells. 8. PMID 31014000 DOI: 10.3390/Cells8040353  0.427
2019 Kmit A, Marson FAL, Pereira SV, Vinagre AM, Leite GS, Servidoni MF, Ribeiro JD, Ribeiro AF, Bertuzzo CS, Amaral MD. Extent of rescue of F508del-CFTR function by VX-809 and VX-770 in human nasal epithelial cells correlates with SNP rs7512462 in SLC26A9 gene in F508del/F508del Cystic Fibrosis patients. Biochimica Et Biophysica Acta. Molecular Basis of Disease. PMID 30716472 DOI: 10.1016/J.Bbadis.2019.01.029  0.32
2019 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. 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. Human Mutation. 40: 326-334. PMID 30488522 DOI: 10.1002/Humu.23692  0.408
2019 Awatade NT, Ramalho S, Silva IAL, Felício V, Botelho HM, de Poel E, Vonk A, Beekman JM, Farinha CM, Amaral MD. R560S: A class II CFTR mutation that is not rescued by current modulators. Journal of Cystic Fibrosis : Official Journal of the European Cystic Fibrosis Society. 18: 182-189. PMID 30030066 DOI: 10.1016/J.Jcf.2018.07.001  0.395
2019 Bene Z, Fejes Z, Fenyvesi F, Váradi J, Clarke LA, Macek M, Amaral MD, Balogh I, Nagy B. WS03-2 Modulation of CFTR alters human epididymis protein 4 (HE4) expression in cystic fibrosis bronchial epithelial cells via NF-κB pathway Journal of Cystic Fibrosis. 18. DOI: 10.1016/S1569-1993(19)30131-6  0.419
2018 Marcão A, Barreto C, Pereira L, Vaz LG, Cavaco J, Casimiro A, Félix M, Silva TR, Barbosa T, Freitas C, Nunes S, Felício V, Lopes L, Amaral M, Vilarinho L. Cystic Fibrosis Newborn Screening in Portugal: PAP Value in Populations with Stringent Rules for Genetic Studies. International Journal of Neonatal Screening. 4: 22. PMID 33072945 DOI: 10.3390/Ijns4030022  0.394
2018 Patel W, Moore PJ, Sassano MF, Lopes-Pacheco M, Aleksandrov AA, Amaral MD, Tarran R, Gray MA. Increases in cytosolic Ca induce dynamin- and calcineurin-dependent internalisation of CFTR. Cellular and Molecular Life Sciences : Cmls. PMID 30547226 DOI: 10.1007/S00018-018-2989-3  0.37
2018 Nagy B, Bene Z, Fejes Z, Heltshe SL, Reid D, Ronan NJ, McCarthy Y, Smith D, Nagy A, Joseloff E, Balla G, Kappelmayer J, Macek M, Bell SC, Plant BJ, ... Amaral MD, et al. Human epididymis protein 4 (HE4) levels inversely correlate with lung function improvement (delta FEV) in cystic fibrosis patients receiving ivacaftor treatment. Journal of Cystic Fibrosis : Official Journal of the European Cystic Fibrosis Society. PMID 30268371 DOI: 10.1016/J.Jcf.2018.08.013  0.39
2018 Matos AM, Gomes-Duarte A, Faria M, Barros P, Jordan P, Amaral MD, Matos P. Prolonged co-treatment with HGF sustains epithelial integrity and improves pharmacological rescue of Phe508del-CFTR. Scientific Reports. 8: 13026. PMID 30158635 DOI: 10.1038/S41598-018-31514-2  0.456
2018 Canato S, Santos JD, Carvalho AS, Aloria K, Amaral MD, Matthiesen R, Falcao AO, Farinha CM. Proteomic interaction profiling reveals KIFC1 as a factor involved in early targeting of F508del-CFTR to degradation. Cellular and Molecular Life Sciences : Cmls. PMID 30066085 DOI: 10.1007/S00018-018-2896-7  0.431
2018 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, et al. CFTR modulator theratyping: Current status, gaps and future directions. Journal of Cystic Fibrosis : Official Journal of the European Cystic Fibrosis Society. PMID 29934203 DOI: 10.1016/J.Jcf.2018.05.004  0.47
2018 Amaral MD, Boj SF, Shaw J, Leipziger J, Beekman JM. Cystic fibrosis: Beyond the airways. Report on the meeting of the basic science working group in Loutraki, Greece. Journal of Cystic Fibrosis : Official Journal of the European Cystic Fibrosis Society. PMID 29866530 DOI: 10.1016/J.Jcf.2018.05.008  0.32
2018 Olivença DV, Uliyakina I, Fonseca LL, Amaral MD, Voit EO, Pinto FR. A Mathematical Model of the Phosphoinositide Pathway. Scientific Reports. 8: 3904. PMID 29500467 DOI: 10.1038/S41598-018-22226-8  0.689
2018 Lérias J, Pinto M, Benedetto R, Schreiber R, Amaral M, Aureli M, Kunzelmann K. Compartmentalized crosstalk of CFTR and TMEM16A (ANO1) through EPAC1 and ADCY1 Cellular Signalling. 44: 10-19. PMID 29331508 DOI: 10.1016/J.Cellsig.2018.01.008  0.376
2018 Liu J, Bihler H, Farinha CM, Awatade NT, Romão AM, Mercadante D, Cheng Y, Musisi I, Jantarajit W, Wang Y, Cai Z, Amaral MD, Mense M, Sheppard DN. Partial rescue of F508del-CFTR channel gating with modest improvement of protein processing, but not stability by a dual-acting small molecule. British Journal of Pharmacology. PMID 29318594 DOI: 10.1111/Bph.14141  0.44
2018 Nagy B, Bene Z, Fejes Z, Heltshe SL, Ronan NJ, Joseloff E, Kappelmayer J, Macek M, Bell SC, Plant BJ, Amaral MD, Balogh I. WS16.6 Human epididymis protein 4 (HE4) plasma levels inversely correlate with improved FEV1 in cystic fibrosis patients under ivacaftor therapy as a new sensitive treatment efficacy biomarker Journal of Cystic Fibrosis. 17. DOI: 10.1016/S1569-1993(18)30213-3  0.346
2018 Silva IAL, Ramalho S, Awatade NT, Centeio R, Farinha CM, Amaral MD. WS15.1 From the bench to bedside: expanding CFTR modulators to rare mutations by response assessment in patients-derived materials and cellular models Journal of Cystic Fibrosis. 17. DOI: 10.1016/S1569-1993(18)30202-9  0.337
2017 Lérias JR, Pinto MC, Botelho HM, Awatade NT, Quaresma MC, Silva IAL, Wanitchakool P, Schreiber R, Pepperkok R, Kunzelmann K, Amaral MD. A novel microscopy-based assay identifies extended synaptotagmin-1 (ESYT1) as a positive regulator of anoctamin 1 traffic. Biochimica Et Biophysica Acta. PMID 29154949 DOI: 10.1016/J.Bbamcr.2017.11.009  0.48
2017 Benedetto R, Ousingsawat J, Wanitchakool P, Zhang Y, Holtzman MJ, Amaral M, Rock JR, Schreiber R, Kunzelmann K. Epithelial Chloride Transport by CFTR Requires TMEM16A. Scientific Reports. 7: 12397. PMID 28963502 DOI: 10.1038/S41598-017-10910-0  0.432
2017 Beekman JM, Wang CM, Casati S, Tuggle KL, Gulmans VAM, Amaral M, De Boeck K. Biobanking: towards increased access of biomaterials in cystic fibrosis. Report on the pre-conference meeting to the 13th ECFS Basic Science Conference, Pisa, 30 March-2 April, 2016. Journal of Cystic Fibrosis : Official Journal of the European Cystic Fibrosis Society. PMID 28478961 DOI: 10.1016/J.Jcf.2017.04.009  0.303
2017 Bosch B, Bilton D, Sosnay P, Raraigh KS, Mak DY, Ishiguro H, Gulmans V, Thomas M, Cuppens H, Amaral M, De Boeck K. Ethnicity impacts the cystic fibrosis diagnosis: A note of caution. Journal of Cystic Fibrosis : Official Journal of the European Cystic Fibrosis Society. PMID 28233695 DOI: 10.1016/J.Jcf.2017.01.016  0.38
2017 Felício V, Ramalho AS, Igreja S, Amaral MD. mRNA-based detection of rare CFTR mutations improves genetic diagnosis of cystic fibrosis in populations with high genetic heterogeneity. Clinical Genetics. 91: 476-481. PMID 27174726 DOI: 10.1111/Cge.12802  0.442
2017 Canato S, Carvalho AS, Botelho HM, Aloria K, Matthiesen R, Amaral MD, Falcão A, Farinha CM. WS09.2 Modulation of protein traffic networks to rescue F508del-CFTR from the endoplasmic reticulum Journal of Cystic Fibrosis. 16. DOI: 10.1016/S1569-1993(17)30208-4  0.307
2016 Amaral MD, Farinha CM, Matos P, Botelho HM. Investigating Alternative Transport of Integral Plasma Membrane Proteins from the ER to the Golgi: Lessons from the Cystic Fibrosis Transmembrane Conductance Regulator (CFTR). Methods in Molecular Biology (Clifton, N.J.). 1459: 105-26. PMID 27665554 DOI: 10.1007/978-1-4939-3804-9_7  0.456
2016 Benedetto R, Sirianant L, Pankonien I, Wanitchakool P, Ousingsawat J, Cabrita I, Schreiber R, Amaral M, Kunzelmann K. Relationship between TMEM16A/anoctamin 1 and LRRC8A. Pflugers Archiv : European Journal of Physiology. PMID 27514381 DOI: 10.1007/S00424-016-1862-1  0.348
2016 Lobo MJ, Amaral MD, Zaccolo M, Farinha CM. EPAC1 activation by cAMP stabilizes CFTR at the membrane by promoting its interaction with NHERF1. Journal of Cell Science. 129: 2599-612. PMID 27206858 DOI: 10.1242/Jcs.185629  0.468
2016 Nagy B, Nagy B, Fila L, Clarke LA, Gönczy F, Bede O, Nagy D, Újhelyi R, Szabó Á, Anghelyi A, Major M, Bene Z, Fejes Z, Antal-Szalmás P, Bhattoa HP, ... ... Amaral MD, et al. Human epididymis protein 4 (HE4): a novel serum inflammatory biomarker in cystic fibrosis. Chest. PMID 27105680 DOI: 10.1016/J.Chest.2016.04.006  0.405
2016 De Boeck K, Amaral MD. Progress in therapies for cystic fibrosis. The Lancet. Respiratory Medicine. PMID 27053340 DOI: 10.1016/S2213-2600(16)00023-0  0.401
2015 Igreja S, Clarke LA, Botelho HM, Marques L, Amaral MD. Correction of a Cystic Fibrosis Splicing Mutation by Antisense Oligonucleotides. Human Mutation. PMID 26553470 DOI: 10.1002/Humu.22931  0.457
2015 Amaral MD, Balch WE. Hallmarks of therapeutic management of the cystic fibrosis functional landscape. Journal of Cystic Fibrosis : Official Journal of the European Cystic Fibrosis Society. PMID 26526359 DOI: 10.1016/J.Jcf.2015.09.006  0.42
2015 Verkman AS, Edelman A, Amaral M, Mall MA, Beekman JM, Meiners T, Galietta LJ, Bear CE. Finding new drugs to enhance anion secretion in cystic fibrosis: Toward suitable systems for better drug screening. Report on the pre-conference meeting to the 12th ECFS Basic Science Conference, Albufeira, 25-28 March 2015. Journal of Cystic Fibrosis : Official Journal of the European Cystic Fibrosis Society. 14: 700-5. PMID 26474804 DOI: 10.1016/J.Jcf.2015.10.001  0.472
2015 Clarke LA, Botelho HM, Sousa L, Falcao AO, Amaral MD. Transcriptome meta-analysis reveals common differential and global gene expression profiles in cystic fibrosis and other respiratory disorders and identifies CFTR regulators. Genomics. 106: 268-77. PMID 26225835 DOI: 10.1016/J.Ygeno.2015.07.005  0.376
2015 Farinha CM, Sousa M, Canato S, Schmidt A, Uliyakina I, Amaral MD. Increased efficacy of VX-809 in different cellular systems results from an early stabilization effect of F508del-CFTR. Pharmacology Research & Perspectives. 3: e00152. PMID 26171232 DOI: 10.1002/Prp2.152  0.494
2015 Awatade NT, Uliyakina I, Farinha CM, Clarke LA, Mendes K, Solé A, Pastor J, Ramos MM, Amaral MD. Measurements of Functional Responses in Human Primary Lung Cells as a Basis for Personalized Therapy for Cystic Fibrosis. Ebiomedicine. 2: 147-53. PMID 26137539 DOI: 10.1016/J.Ebiom.2014.12.005  0.452
2015 Hartl D, Amaral M. Cystic fibrosis -- From basic science to clinical benefit: A review series. Journal of Cystic Fibrosis. 14: 415-416. PMID 26088670 DOI: 10.1016/J.Jcf.2015.06.001  0.306
2015 Loureiro CA, Matos AM, Dias-Alves Â, Pereira JF, Uliyakina I, Barros P, Amaral MD, Matos P. A molecular switch in the scaffold NHERF1 enables misfolded CFTR to evade the peripheral quality control checkpoint. Science Signaling. 8: ra48. PMID 25990958 DOI: 10.1126/Scisignal.Aaa1580  0.463
2015 Botelho HM, Uliyakina I, Awatade NT, Proença MC, Tischer C, Sirianant L, Kunzelmann K, Pepperkok R, Amaral MD. Protein traffic disorders: an effective high-throughput fluorescence microscopy pipeline for drug discovery. Scientific Reports. 5: 9038. PMID 25762484 DOI: 10.1038/Srep09038  0.361
2015 Ramalho AS, Clarke LA, Sousa M, Felicio V, Barreto C, Lopes C, Amaral MD. Comparative ex vivo, in vitro and in silico analyses of a CFTR splicing mutation: Importance of functional studies to establish disease liability of mutations. Journal of Cystic Fibrosis : Official Journal of the European Cystic Fibrosis Society. PMID 25735457 DOI: 10.1016/J.Jcf.2015.02.002  0.458
2015 Amaral MD. Novel personalized therapies for cystic fibrosis: treating the basic defect in all patients. Journal of Internal Medicine. 277: 155-66. PMID 25266997 DOI: 10.1111/Joim.12314  0.455
2015 Bell SC, De Boeck K, Amaral MD. New pharmacological approaches for cystic fibrosis: promises, progress, pitfalls. Pharmacology & Therapeutics. 145: 19-34. PMID 24932877 DOI: 10.1016/J.Pharmthera.2014.06.005  0.439
2015 Srivastava JK, Awatade NT, Bhat HR, Kmit A, Mendes K, Ramos M, Amaral MD, Singh UP. Pharmacological evaluation of hybrid thiazolidin-4-one-1,3,5-triazines for NF-κB, biofilm and CFTR activity Rsc Advances. 5: 88710-88718. DOI: 10.1039/C5Ra09250G  0.394
2015 Lobo M, Fernandes J, Amaral M, Zaccolo M, Farinha CM. WS06.1 Stabilization of CFTR at the membrane by EPAC1 activation occurs through interaction with NHERF-1 Journal of Cystic Fibrosis. 14. DOI: 10.1016/S1569-1993(15)30033-3  0.455
2014 Sharma N, Sosnay PR, Ramalho AS, Douville C, Franca A, Gottschalk LB, Park J, Lee M, Vecchio-Pagan B, Raraigh KS, Amaral MD, Karchin R, Cutting GR. Experimental assessment of splicing variants using expression minigenes and comparison with in silico predictions. Human Mutation. 35: 1249-59. PMID 25066652 DOI: 10.1002/Humu.22624  0.386
2014 Luz S, Cihil KM, Brautigan DL, Amaral MD, Farinha CM, Swiatecka-Urban A. LMTK2-mediated phosphorylation regulates CFTR endocytosis in human airway epithelial cells. The Journal of Biological Chemistry. 289: 15080-93. PMID 24727471 DOI: 10.1074/Jbc.M114.563742  0.467
2014 Xu Z, Pissarra LS, Farinha CM, Liu J, Cai Z, Thibodeau PH, Amaral MD, Sheppard DN. Revertant mutants modify, but do not rescue, the gating defect of the cystic fibrosis mutant G551D-CFTR. The Journal of Physiology. 592: 1931-47. PMID 24591578 DOI: 10.1113/Jphysiol.2014.271817  0.477
2014 Masvidal L, Igreja S, Ramos MD, Alvarez A, de Gracia J, Ramalho A, Amaral MD, Larriba S, Casals T. Assessing the residual CFTR gene expression in human nasal epithelium cells bearing CFTR splicing mutations causing cystic fibrosis. European Journal of Human Genetics : Ejhg. 22: 784-91. PMID 24129438 DOI: 10.1038/Ejhg.2013.238  0.436
2013 Almaça J, Faria D, Sousa M, Uliyakina I, Conrad C, Sirianant L, Clarke LA, Martins JP, Santos M, Heriché JK, Huber W, Schreiber R, Pepperkok R, Kunzelmann K, Amaral MD. High-content siRNA screen reveals global ENaC regulators and potential cystic fibrosis therapy targets. Cell. 154: 1390-400. PMID 24034256 DOI: 10.1016/J.Cell.2013.08.045  0.493
2013 Silva MC, Amaral MD, Morimoto RI. Neuronal reprograming of protein homeostasis by calcium-dependent regulation of the heat shock response. Plos Genetics. 9: e1003711. PMID 24009518 DOI: 10.1371/Journal.Pgen.1003711  0.33
2013 Sosnay PR, Siklosi KR, Van Goor F, Kaniecki K, Yu H, Sharma N, Ramalho AS, Amaral MD, Dorfman R, Zielenski J, Masica DL, Karchin R, Millen L, Thomas PJ, Patrinos GP, et al. Defining the disease liability of variants in the cystic fibrosis transmembrane conductance regulator gene. Nature Genetics. 45: 1160-7. PMID 23974870 DOI: 10.1038/Ng.2745  0.38
2013 Farinha CM, King-Underwood J, Sousa M, Correia AR, Henriques BJ, Roxo-Rosa M, Da Paula AC, Williams J, Hirst S, Gomes CM, Amaral MD. Revertants, low temperature, and correctors reveal the mechanism of F508del-CFTR rescue by VX-809 and suggest multiple agents for full correction. Chemistry & Biology. 20: 943-55. PMID 23890012 DOI: 10.1016/J.Chembiol.2013.06.004  0.437
2013 Farinha CM, Matos P, Amaral MD. Control of cystic fibrosis transmembrane conductance regulator membrane trafficking: not just from the endoplasmic reticulum to the Golgi. The Febs Journal. 280: 4396-406. PMID 23773658 DOI: 10.1111/Febs.12392  0.472
2013 Servidoni MF, Sousa M, Vinagre AM, Cardoso SR, Ribeiro MA, Meirelles LR, de Carvalho RB, Kunzelmann K, Ribeiro AF, Ribeiro JD, Amaral MD. Rectal forceps biopsy procedure in cystic fibrosis: technical aspects and patients perspective for clinical trials feasibility. Bmc Gastroenterology. 13: 91. PMID 23688510 DOI: 10.1186/1471-230X-13-91  0.321
2013 Amaral MD, Farinha CM. Post-translational modifications of CFTR: insight into protein trafficking and cystic fibrosis disease. The Febs Journal. 280: 4395. PMID 23680006 DOI: 10.1111/Febs.12345  0.414
2013 Clarke LA, Sousa L, Barreto C, Amaral MD. Changes in transcriptome of native nasal epithelium expressing F508del-CFTR and intersecting data from comparable studies. Respiratory Research. 14: 38. PMID 23537407 DOI: 10.1186/1465-9921-14-38  0.449
2013 Amaral MD, Farinha CM. Rescuing mutant CFTR: a multi-task approach to a better outcome in treating cystic fibrosis. Current Pharmaceutical Design. 19: 3497-508. PMID 23331027 DOI: 10.2174/13816128113199990318  0.429
2013 Moniz S, Sousa M, Moraes BJ, Mendes AI, Palma M, Barreto C, Fragata JI, Amaral MD, Matos P. HGF stimulation of Rac1 signaling enhances pharmacological correction of the most prevalent cystic fibrosis mutant F508del-CFTR. Acs Chemical Biology. 8: 432-42. PMID 23148778 DOI: 10.1021/Cb300484R  0.509
2013 Tosoni K, Stobbart M, Cassidy DM, Venerando A, Pagano MA, Luz S, Amaral MD, Kunzelmann K, Pinna LA, Farinha CM, Mehta A. CFTR mutations altering CFTR fragmentation. The Biochemical Journal. 449: 295-305. PMID 23067305 DOI: 10.1042/Bj20121240  0.426
2013 Tian Y, Schreiber R, Wanitchakool P, Kongsuphol P, Sousa M, Uliyakina I, Palma M, Faria D, Traynor-Kaplan AE, Fragata JI, Amaral MD, Kunzelmann K. Control of TMEM16A by INO-4995 and other inositolphosphates. British Journal of Pharmacology. 168: 253-65. PMID 22946960 DOI: 10.1111/J.1476-5381.2012.02193.X  0.332
2013 De Boeck K, Kent L, Davies J, Derichs N, Amaral M, Rowe SM, Middleton P, de Jonge H, Bronsveld I, Wilschanski M, Melotti P, Danner-Boucher I, Boerner S, Fajac I, Southern K, et al. CFTR biomarkers: time for promotion to surrogate end-point. The European Respiratory Journal. 41: 203-16. PMID 22878883 DOI: 10.1183/09031936.00057512  0.341
2013 Canato S, Telhada M, Amaral M, Clarke L. WS22.5 Effect of the Burkholderia cenocepacia exoproteome on CFTR protein expression in human cystic fibrosis lung epithelial cell models Journal of Cystic Fibrosis. 12: S47. DOI: 10.1016/S1569-1993(13)60142-3  0.304
2013 Dahimène S, Romão A, Botelho HM, Palma M, Correia AR, Farinha CM, King-Underwood J, Williams J, Hirst S, Gomes CM, Amaral MD. WS21.9 Screening for novel F508del-CFTR correctors identified by pharmacophore modelling through automated microscopy and NBD1 folding assays Journal of Cystic Fibrosis. 12. DOI: 10.1016/S1569-1993(13)60137-X  0.347
2013 Botelho HM, Dahimène S, Uliyakina I, Neumann B, Tischer C, Pepperkok R, Amaral MD. WS20.6 High throughput screening as a source for novel cystic fibrosis therapeutic targets Journal of Cystic Fibrosis. 12. DOI: 10.1016/S1569-1993(13)60128-9  0.408
2013 Clarke LA, Sousa L, Amaral MD. WS20.3 Transcriptomics of native nasal epithelium expressing F508del-CFTR and intersecting data from comparable studies Journal of Cystic Fibrosis. 12. DOI: 10.1016/S1569-1993(13)60125-3  0.325
2012 Mendes F, Farinha CM, Felício V, Alves PC, Vieira I, Amaral MD. BAG-1 stabilizes mutant F508del-CFTR in a ubiquitin-like-domain-dependent manner. Cellular Physiology and Biochemistry : International Journal of Experimental Cellular Physiology, Biochemistry, and Pharmacology. 30: 1120-33. PMID 23178238 DOI: 10.1159/000343303  0.447
2012 Sousa M, Servidoni MF, Vinagre AM, Ramalho AS, Bonadia LC, Felício V, Ribeiro MA, Uliyakina I, Marson FA, Kmit A, Cardoso SR, Ribeiro JD, Bertuzzo CS, Sousa L, Kunzelmann K, ... ... Amaral MD, et al. Measurements of CFTR-mediated Cl- secretion in human rectal biopsies constitute a robust biomarker for Cystic Fibrosis diagnosis and prognosis. Plos One. 7: e47708. PMID 23082198 DOI: 10.1371/Journal.Pone.0047708  0.483
2012 Li H, Yang W, Mendes F, Amaral MD, Sheppard DN. Impact of the cystic fibrosis mutation F508del-CFTR on renal cyst formation and growth. American Journal of Physiology. Renal Physiology. 303: F1176-86. PMID 22874761 DOI: 10.1152/Ajprenal.00130.2012  0.454
2012 Faria D, Lentze N, Almaça J, Luz S, Alessio L, Tian Y, Martins JP, Cruz P, Schreiber R, Rezwan M, Farinha CM, Auerbach D, Amaral MD, Kunzelmann K. Regulation of ENaC biogenesis by the stress response protein SERP1. Pflã¼Gers Archiv : European Journal of Physiology. 463: 819-27. PMID 22526458 DOI: 10.1007/S00424-012-1091-1  0.438
2012 Amaral MD. Finding new medicines to fight CF: multiple steps of a success story. Orphanet Journal of Rare Diseases. 7: 1-2. DOI: 10.1186/1750-1172-7-S2-A19  0.462
2011 Silva MC, Fox S, Beam M, Thakkar H, Amaral MD, Morimoto RI. A genetic screening strategy identifies novel regulators of the proteostasis network. Plos Genetics. 7: e1002438. PMID 22242008 DOI: 10.1371/Journal.Pgen.1002438  0.34
2011 Luz S, Kongsuphol P, Mendes AI, Romeiras F, Sousa M, Schreiber R, Matos P, Jordan P, Mehta A, Amaral MD, Kunzelmann K, Farinha CM. Contribution of casein kinase 2 and spleen tyrosine kinase to CFTR trafficking and protein kinase A-induced activity. Molecular and Cellular Biology. 31: 4392-404. PMID 21930781 DOI: 10.1128/Mcb.05517-11  0.449
2011 Roth EK, Hirtz S, Duerr J, Wenning D, Eichler I, Seydewitz HH, Amaral MD, Mall MA. The K+ channel opener 1-EBIO potentiates residual function of mutant CFTR in rectal biopsies from cystic fibrosis patients. Plos One. 6: e24445. PMID 21909392 DOI: 10.1371/Journal.Pone.0024445  0.447
2011 Martins JR, Kongsuphol P, Sammels E, Dahimène S, Aldehni F, Clarke LA, Schreiber R, de Smedt H, Amaral MD, Kunzelmann K. F508del-CFTR increases intracellular Ca(2+) signaling that causes enhanced calcium-dependent Cl(-) conductance in cystic fibrosis. Biochimica Et Biophysica Acta. 1812: 1385-92. PMID 21907281 DOI: 10.1016/J.Bbadis.2011.08.008  0.383
2011 Mendes AI, Matos P, Moniz S, Luz S, Amaral MD, Farinha CM, Jordan P. Antagonistic regulation of cystic fibrosis transmembrane conductance regulator cell surface expression by protein kinases WNK4 and spleen tyrosine kinase. Molecular and Cellular Biology. 31: 4076-86. PMID 21807898 DOI: 10.1128/Mcb.05152-11  0.432
2011 Amaral MD, Lukacs GL. Introduction to section III: biochemical methods to study CFTR protein. Methods in Molecular Biology (Clifton, N.J.). 741: 213-8. PMID 21594787 DOI: 10.1007/978-1-61779-117-8_14  0.418
2011 Ramalho AS, Clarke LA, Amaral MD. Quantification of CFTR transcripts. Methods in Molecular Biology (Clifton, N.J.). 741: 115-35. PMID 21594782 DOI: 10.1007/978-1-61779-117-8_9  0.376
2011 Amaral MD. Introduction to section III: resources for CFTR research. Methods in Molecular Biology (Clifton, N.J.). 742: 281-3. PMID 21547739 DOI: 10.1007/978-1-61779-120-8_17  0.324
2011 Almaça J, Dahimène S, Appel N, Conrad C, Kunzelmann K, Pepperkok R, Amaral MD. Functional genomics assays to study CFTR traffic and ENaC function. Methods in Molecular Biology (Clifton, N.J.). 742: 249-64. PMID 21547737 DOI: 10.1007/978-1-61779-120-8_15  0.371
2011 Ramachandran S, Clarke LA, Scheetz TE, Amaral MD, McCray PB. Microarray mRNA expression profiling to study cystic fibrosis. Methods in Molecular Biology (Clifton, N.J.). 742: 193-212. PMID 21547734 DOI: 10.1007/978-1-61779-120-8_12  0.436
2011 Faria D, Dahimène S, Alessio L, Scott-Ward T, Schreiber R, Kunzelmann K, Amaral MD. Effect of Annexin A5 on CFTR: regulated traffic or scaffolding? Molecular Membrane Biology. 28: 14-29. PMID 21067452 DOI: 10.3109/09687688.2010.506264  0.362
2011 Amaral MD. Targeting CFTR: how to treat cystic fibrosis by CFTR-repairing therapies. Current Drug Targets. 12: 683-93. PMID 21039334 DOI: 10.2174/138945011795378586  0.505
2010 Da Paula AC, Sousa M, Xu Z, Dawson ES, Boyd AC, Sheppard DN, Amaral MD. Folding and rescue of a cystic fibrosis transmembrane conductance regulator trafficking mutant identified using human-murine chimeric proteins. The Journal of Biological Chemistry. 285: 27033-44. PMID 20551307 DOI: 10.1074/Jbc.M110.120352  0.466
2010 Rocchi L, Braz C, Cattani S, Ramalho A, Christan S, Edlinger M, Ascenzioni F, Laner A, Kraner S, Amaral M, Schindelhauer D. Escherichia coli-cloned CFTR loci relevant for human artificial chromosome therapy. Human Gene Therapy. 21: 1077-92. PMID 20384480 DOI: 10.1089/Hum.2009.225  0.325
2009 Ramalho AS, Lewandowska MA, Farinha CM, Mendes F, Gonçalves J, Barreto C, Harris A, Amaral MD. Deletion of CFTR translation start site reveals functional isoforms of the protein in CF patients. Cellular Physiology and Biochemistry : International Journal of Experimental Cellular Physiology, Biochemistry, and Pharmacology. 24: 335-46. PMID 19910674 DOI: 10.1159/000257426  0.445
2009 Scott-Ward TS, Amaral MD. Deletion of Phe508 in the first nucleotide-binding domain of the cystic fibrosis transmembrane conductance regulator increases its affinity for the heat shock cognate 70 chaperone. The Febs Journal. 276: 7097-109. PMID 19878303 DOI: 10.1111/J.1742-4658.2009.07421.X  0.394
2009 Almaça J, Kongsuphol P, Hieke B, Ousingsawat J, Viollet B, Schreiber R, Amaral MD, Kunzelmann K. AMPK controls epithelial Na(+) channels through Nedd4-2 and causes an epithelial phenotype when mutated. Pflã¼Gers Archiv : European Journal of Physiology. 458: 713-21. PMID 19333618 DOI: 10.1007/S00424-009-0660-4  0.336
2008 Bachhuber T, Almaça J, Aldehni F, Mehta A, Amaral MD, Schreiber R, Kunzelmann K. Regulation of the epithelial Na+ channel by the protein kinase CK2. The Journal of Biological Chemistry. 283: 13225-32. PMID 18308722 DOI: 10.1074/Jbc.M704532200  0.391
2008 Schmidt A, Hughes LK, Cai Z, Mendes F, Li H, Sheppard DN, Amaral MD. Prolonged treatment of cells with genistein modulates the expression and function of the cystic fibrosis transmembrane conductance regulator. British Journal of Pharmacology. 153: 1311-23. PMID 18223673 DOI: 10.1038/Sj.Bjp.0707663  0.471
2008 Pissarra LS, Farinha CM, Xu Z, Schmidt A, Thibodeau PH, Cai Z, Thomas PJ, Sheppard DN, Amaral MD. Solubilizing mutations used to crystallize one CFTR domain attenuate the trafficking and channel defects caused by the major cystic fibrosis mutation. Chemistry & Biology. 15: 62-9. PMID 18215773 DOI: 10.1016/J.Chembiol.2007.11.012  0.423
2008 Rakonczay Z, Hegyi P, Hasegawa M, Inoue M, You J, Iida A, Ignáth I, Alton EW, Griesenbach U, Ovári G, Vág J, Da Paula AC, Crawford RM, Varga G, Amaral MD, et al. CFTR gene transfer to human cystic fibrosis pancreatic duct cells using a Sendai virus vector. Journal of Cellular Physiology. 214: 442-55. PMID 17654517 DOI: 10.1002/Jcp.21220  0.455
2007 Garcia SM, Casanueva MO, Silva MC, Amaral MD, Morimoto RI. Neuronal signaling modulates protein homeostasis in Caenorhabditis elegans post-synaptic muscle cells. Genes & Development. 21: 3006-16. PMID 18006691 DOI: 10.1101/Gad.1575307  0.32
2007 Scott-Ward TS, Cai Z, Dawson ES, Doherty A, Da Paula AC, Davidson H, Porteous DJ, Wainwright BJ, Amaral MD, Sheppard DN, Boyd AC. Chimeric constructs endow the human CFTR Cl- channel with the gating behavior of murine CFTR. Proceedings of the National Academy of Sciences of the United States of America. 104: 16365-70. PMID 17913891 DOI: 10.1073/Pnas.0701562104  0.382
2007 Amaral MD, Kunzelmann K. Molecular targeting of CFTR as a therapeutic approach to cystic fibrosis. Trends in Pharmacological Sciences. 28: 334-41. PMID 17573123 DOI: 10.1016/J.Tips.2007.05.004  0.421
2007 Sousa M, Ousingsawat J, Seitz R, Puntheeranurak S, Regalado A, Schmidt A, Grego T, Jansakul C, Amaral MD, Schreiber R, Kunzelmann K. An extract from the medicinal plant Phyllanthus acidus and its isolated compounds induce airway chloride secretion: A potential treatment for cystic fibrosis. Molecular Pharmacology. 71: 366-76. PMID 17065237 DOI: 10.1124/Mol.106.025262  0.451
2006 Roxo-Rosa M, Xu Z, Schmidt A, Neto M, Cai Z, Soares CM, Sheppard DN, Amaral MD. Revertant mutants G550E and 4RK rescue cystic fibrosis mutants in the first nucleotide-binding domain of CFTR by different mechanisms. Proceedings of the National Academy of Sciences of the United States of America. 103: 17891-6. PMID 17098864 DOI: 10.1073/Pnas.0608312103  0.478
2006 Amaral MD. Therapy through chaperones: sense or antisense? Cystic fibrosis as a model disease. Journal of Inherited Metabolic Disease. 29: 477-87. PMID 16763920 DOI: 10.1007/S10545-006-0251-X  0.411
2006 Roxo-Rosa M, da Costa G, Luider TM, Scholte BJ, Coelho AV, Amaral MD, Penque D. Proteomic analysis of nasal cells from cystic fibrosis patients and non-cystic fibrosis control individuals: search for novel biomarkers of cystic fibrosis lung disease. Proteomics. 6: 2314-25. PMID 16518875 DOI: 10.1002/Pmic.200500273  0.465
2006 Davidson H, McLachlan G, Wilson A, Boyd AC, Doherty A, MacGregor G, Davies L, Painter HA, Coles R, Hyde SC, Gill DR, Amaral MD, Collie DD, Porteous DJ, Penque D. Human-specific cystic fibrosis transmembrane conductance regulator antibodies detect in vivo gene transfer to ovine airways. American Journal of Respiratory Cell and Molecular Biology. 35: 72-83. PMID 16498081 DOI: 10.1165/Rcmb.2005-0377Oc  0.384
2005 Mendes F, Wakefield J, Bachhuber T, Barroso M, Bebok Z, Penque D, Kunzelmann K, Amaral MD. Establishment and characterization of a novel polarized MDCK epithelial cellular model for CFTR studies. Cellular Physiology and Biochemistry : International Journal of Experimental Cellular Physiology, Biochemistry, and Pharmacology. 16: 281-90. PMID 16301828 DOI: 10.1159/000089857  0.487
2005 da Paula AC, Ramalho AS, Farinha CM, Cheung J, Maurisse R, Gruenert DC, Ousingsawat J, Kunzelmann K, Amaral MD. Characterization of novel airway submucosal gland cell models for cystic fibrosis studies. Cellular Physiology and Biochemistry : International Journal of Experimental Cellular Physiology, Biochemistry, and Pharmacology. 15: 251-62. PMID 16037690 DOI: 10.1159/000087235  0.445
2005 Farinha CM, Amaral MD. Most F508del-CFTR is targeted to degradation at an early folding checkpoint and independently of calnexin. Molecular and Cellular Biology. 25: 5242-52. PMID 15923638 DOI: 10.1128/Mcb.25.12.5242-5252.2005  0.449
2005 Englmann A, Clarke LA, Christan S, Amaral MD, Schindelhauer D, Zink D. The replication timing of CFTR and adjacent genes. Chromosome Research : An International Journal On the Molecular, Supramolecular and Evolutionary Aspects of Chromosome Biology. 13: 183-94. PMID 15861307 DOI: 10.1007/S10577-005-0845-4  0.326
2005 Amaral MD. Processing of CFTR: traversing the cellular maze--how much CFTR needs to go through to avoid cystic fibrosis? Pediatric Pulmonology. 39: 479-91. PMID 15765539 DOI: 10.1002/Ppul.20168  0.485
2004 Hirtz S, Gonska T, Seydewitz HH, Thomas J, Greiner P, Kuehr J, Brandis M, Eichler I, Rocha H, Lopes AI, Barreto C, Ramalho A, Amaral MD, Kunzelmann K, Mall M. CFTR Cl- channel function in native human colon correlates with the genotype and phenotype in cystic fibrosis. Gastroenterology. 127: 1085-95. PMID 15480987 DOI: 10.1053/J.Gastro.2004.07.006  0.497
2004 Roxo-Rosa M, Davezac N, Bensalem N, Majumder M, Heda GD, Simas A, Penque D, Amaral MD, Lukacs GL, Edelman A. Proteomics techniques for cystic fibrosis research. Journal of Cystic Fibrosis : Official Journal of the European Cystic Fibrosis Society. 3: 85-9. PMID 15463934 DOI: 10.1016/J.Jcf.2004.05.019  0.495
2004 Farinha CM, Penque D, Roxo-Rosa M, Lukacs G, Dormer R, McPherson M, Pereira M, Bot AG, Jorna H, Willemsen R, Dejonge H, Heda GD, Marino CR, Fanen P, Hinzpeter A, ... ... Amaral MD, et al. Biochemical methods to assess CFTR expression and membrane localization. Journal of Cystic Fibrosis : Official Journal of the European Cystic Fibrosis Society. 3: 73-7. PMID 15463932 DOI: 10.1016/J.Jcf.2004.05.017  0.397
2004 Mendes F, Farinha CM, Roxo-Rosa M, Fanen P, Edelman A, Dormer R, McPherson M, Davidson H, Puchelle E, De Jonge H, Heda GD, Gentzsch M, Lukacs G, Penque D, Amaral MD. Antibodies for CFTR studies. Journal of Cystic Fibrosis : Official Journal of the European Cystic Fibrosis Society. 3: 69-72. PMID 15463931 DOI: 10.1016/J.Jcf.2004.05.016  0.406
2004 Harris CM, Mendes F, Dragomir A, Doull IJ, Carvalho-Oliveira I, Bebok Z, Clancy JP, Eubanks V, Sorscher EJ, Roomans GM, Amaral MD, McPherson MA, Penque D, Dormer RL. Assessment of CFTR localisation in native airway epithelial cells obtained by nasal brushing. Journal of Cystic Fibrosis : Official Journal of the European Cystic Fibrosis Society. 3: 43-8. PMID 15463924 DOI: 10.1016/J.Jcf.2004.05.009  0.432
2004 Mendes F, Doucet L, Hinzpeter A, Férec C, Lipecka J, Fritsch J, Edelman A, Jorna H, Willemsen R, Bot AG, De Jonge HR, Hinnrasky J, Castillon N, Taouil K, Puchelle E, ... ... Amaral MD, et al. Immunohistochemistry of CFTR in native tissues and primary epithelial cell cultures. Journal of Cystic Fibrosis : Official Journal of the European Cystic Fibrosis Society. 3: 37-41. PMID 15463923 DOI: 10.1016/J.Jcf.2004.05.008  0.364
2004 Galvin P, Clarke LA, Harvey S, Amaral MD. Microarray analysis in cystic fibrosis. Journal of Cystic Fibrosis : Official Journal of the European Cystic Fibrosis Society. 3: 29-33. PMID 15463921 DOI: 10.1016/J.Jcf.2004.05.006  0.408
2004 Trezise AE, Farinha CM, Heda GD, Harris A, Amaral MD, Mouchel N. Non-PCR methods for the analysis of CFTR transcripts. Journal of Cystic Fibrosis : Official Journal of the European Cystic Fibrosis Society. 3: 25-8. PMID 15463920 DOI: 10.1016/J.Jcf.2004.05.005  0.392
2004 Amaral MD, Clarke LA, Ramalho AS, Beck S, Broackes-Carter F, Rowntree R, Mouchel N, Williams SH, Harris A, Tzetis M, Steiner B, Sanz J, Gallati S, Nissim-Rafinifa M, Kerem B, et al. Quantitative methods for the analysis of CFTR transcripts/splicing variants. Journal of Cystic Fibrosis : Official Journal of the European Cystic Fibrosis Society. 3: 17-23. PMID 15463919 DOI: 10.1016/J.Jcf.2004.05.047  0.367
2004 Ramalho AS, Beck S, Farinha CM, Clarke LA, Heda GD, Steiner B, Sanz J, Gallati S, Amaral MD, Harris A, Tzetis M. Methods for RNA extraction, cDNA preparation and analysis of CFTR transcripts. Journal of Cystic Fibrosis : Official Journal of the European Cystic Fibrosis Society. 3: 11-5. PMID 15463918 DOI: 10.1016/J.Jcf.2004.05.004  0.321
2004 Férec C, Le Maréchal C, Audrézet MP, Farinha CM, Amaral MD, Gallati S, Sanz J, Steiner B, Mouchel N, Harris A, Schwarz MJ. Analysis of genomic CFTR DNA. Journal of Cystic Fibrosis : Official Journal of the European Cystic Fibrosis Society. 3: 7-10. PMID 15463917 DOI: 10.1016/J.Jcf.2004.05.003  0.347
2004 Zink D, Amaral MD, Englmann A, Lang S, Clarke LA, Rudolph C, Alt F, Luther K, Braz C, Sadoni N, Rosenecker J, Schindelhauer D. Transcription-dependent spatial arrangements of CFTR and adjacent genes in human cell nuclei. The Journal of Cell Biology. 166: 815-25. PMID 15364959 DOI: 10.1083/Jcb.200404107  0.303
2004 Farinha CM, Mendes F, Roxo-Rosa M, Penque D, Amaral MD. A comparison of 14 antibodies for the biochemical detection of the cystic fibrosis transmembrane conductance regulator protein. Molecular and Cellular Probes. 18: 235-42. PMID 15271383 DOI: 10.1016/J.Mcp.2004.03.005  0.429
2004 Amaral MD. CFTR and chaperones: processing and degradation. Journal of Molecular Neuroscience : Mn. 23: 41-8. PMID 15126691 DOI: 10.1385/JMN:23:1-2:041  0.411
2004 Barreto C, Mall M, Amaral MD. Assessment of CFTR function in native epithelia for the diagnosis of cystic fibrosis. Pediatric Pulmonology. Supplement. 26: 243. PMID 15029663 DOI: 10.1002/Ppul.70118  0.378
2004 Carvalho-Oliveira I, Efthymiadou A, Malhó R, Nogueira P, Tzetis M, Kanavakis E, Amaral MD, Penque D. CFTR localization in native airway cells and cell lines expressing wild-type or F508del-CFTR by a panel of different antibodies. The Journal of Histochemistry and Cytochemistry : Official Journal of the Histochemistry Society. 52: 193-203. PMID 14729871 DOI: 10.1177/002215540405200207  0.455
2004 Maurício AC, Penque D, Amaral MD, Ferreira KT. Ionic transport in tall columnar epithelial (TCE) cells obtained by nasal brushing from non-cystic fibrosis (CF) individuals. Acta MéDica Portuguesa. 17: 427-434. DOI: 10.20344/Amp.1123  0.402
2004 Edelman A, Amaral MD. General introduction to section C: Biochemistry and Biophysics of CFTR Journal of Cystic Fibrosis. 3: 67. DOI: 10.1016/J.Jcf.2004.05.015  0.487
2003 Mendes F, Roxo Rosa M, Dragomir A, Farinha CM, Roomans GM, Amaral MD, Penque D. Unusually common cystic fibrosis mutation in Portugal encodes a misprocessed protein. Biochemical and Biophysical Research Communications. 311: 665-71. PMID 14623323 DOI: 10.1016/J.Bbrc.2003.10.048  0.438
2003 Doucet L, Mendes F, Montier T, Delépine P, Penque D, Férec C, Amaral MD. Applicability of different antibodies for the immunohistochemical localization of CFTR in respiratory and intestinal tissues of human and murine origin. The Journal of Histochemistry and Cytochemistry : Official Journal of the Histochemistry Society. 51: 1191-9. PMID 12923244 DOI: 10.1177/002215540305100909  0.443
2003 Ramalho AS, Beck S, Penque D, Gonska T, Seydewitz HH, Mall M, Amaral MD. Transcript analysis of the cystic fibrosis splicing mutation 1525-1G>A shows use of multiple alternative splicing sites and suggests a putative role of exonic splicing enhancers. Journal of Medical Genetics. 40: e88. PMID 12843337 DOI: 10.1136/Jmg.40.7.E88  0.451
2002 Ramalho AS, Beck S, Meyer M, Penque D, Cutting GR, Amaral MD. Five percent of normal cystic fibrosis transmembrane conductance regulator mRNA ameliorates the severity of pulmonary disease in cystic fibrosis. American Journal of Respiratory Cell and Molecular Biology. 27: 619-27. PMID 12397022 DOI: 10.1165/Rcmb.2001-0004Oc  0.421
2002 Farinha CM, Nogueira P, Mendes F, Penque D, Amaral MD. The human DnaJ homologue (Hdj)-1/heat-shock protein (Hsp) 40 co-chaperone is required for the in vivo stabilization of the cystic fibrosis transmembrane conductance regulator by Hsp70. The Biochemical Journal. 366: 797-806. PMID 12069690 DOI: 10.1042/Bj20011717  0.415
2000 Penque D, Mendes F, Beck S, Farinha C, Pacheco P, Nogueira P, Lavinha J, Malhó R, Amaral MD. Cystic fibrosis F508del patients have apically localized CFTR in a reduced number of airway cells. Laboratory Investigation; a Journal of Technical Methods and Pathology. 80: 857-68. PMID 10879737 DOI: 10.1038/Labinvest.3780090  0.45
1999 Beck S, Penque D, Garcia S, Gomes A, Farinha C, Mata L, Gulbenkian S, Gil-Ferreira K, Duarte A, Pacheco P, Barreto C, Lopes B, Cavaco J, Lavinha J, Amaral MD. Cystic fibrosis patients with the 3272-26A-->G mutation have mild disease, leaky alternative mRNA splicing, and CFTR protein at the cell membrane. Human Mutation. 14: 133-44. PMID 10425036 DOI: 10.1002/(Sici)1098-1004(1999)14:2<133::Aid-Humu5>3.0.Co;2-T  0.463
1996 Duarte A, Amaral M, Barreto C, Pacheco P, Lavinha J. Complex cystic fibrosis allele R334W-R1158X results in reduced levels of correctly processed mRNA in a pancreatic sufficient patient. Human Mutation. 8: 134-9. PMID 8844211 DOI: 10.1002/(Sici)1098-1004(1996)8:2<134::Aid-Humu5>3.0.Co;2-B  0.415
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