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Items: 42

1.

Network Biology Identifies Novel Regulators of CFTR Trafficking and Membrane Stability.

Loureiro CA, Santos JD, Matos AM, Jordan P, Matos P, Farinha CM, Pinto FR.

Front Pharmacol. 2019 Jun 4;10:619. doi: 10.3389/fphar.2019.00619. eCollection 2019.

2.

Omics in CF - Where are we now?

Farinha CM.

J Cyst Fibros. 2019 Jul;18(4):443-444. doi: 10.1016/j.jcf.2019.05.016. Epub 2019 May 31. No abstract available.

PMID:
31155455
3.

Folding Status Is Determinant over Traffic-Competence in Defining CFTR Interactors in the Endoplasmic Reticulum.

Santos JD, Canato S, Carvalho AS, Botelho HM, Aloria K, Amaral MD, Matthiesen R, Falcao AO, Farinha CM.

Cells. 2019 Apr 14;8(4). pii: E353. doi: 10.3390/cells8040353.

4.

Unraveling the Function of Lemur Tyrosine Kinase 2 Network.

Cruz DF, Farinha CM, Swiatecka-Urban A.

Front Pharmacol. 2019 Jan 29;10:24. doi: 10.3389/fphar.2019.00024. eCollection 2019. Review.

5.

Proteomic interaction profiling reveals KIFC1 as a factor involved in early targeting of F508del-CFTR to degradation.

Canato S, Santos JD, Carvalho AS, Aloria K, Amaral MD, Matthiesen R, Falcao AO, Farinha CM.

Cell Mol Life Sci. 2018 Dec;75(24):4495-4509. doi: 10.1007/s00018-018-2896-7. Epub 2018 Jul 31.

PMID:
30066085
6.

R560S: A class II CFTR mutation that is not rescued by current modulators.

Awatade NT, Ramalho S, Silva IAL, Felício V, Botelho HM, de Poel E, Vonk A, Beekman JM, Farinha CM, Amaral MD.

J Cyst Fibros. 2019 Mar;18(2):182-189. doi: 10.1016/j.jcf.2018.07.001. Epub 2018 Jul 18.

PMID:
30030066
7.

Targeting of the Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) Protein with a Technetium-99m Imaging Probe.

Ferreira VFC, Oliveira BL, Santos JD, Correia JDG, Farinha CM, Mendes F.

ChemMedChem. 2018 Jul 18;13(14):1469-1478. doi: 10.1002/cmdc.201800187. Epub 2018 Jun 12.

PMID:
29864241
8.

Partial rescue of F508del-cystic fibrosis transmembrane conductance regulator channel gating with modest improvement of protein processing, but not stability, by a dual-acting small molecule.

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.

Br J Pharmacol. 2018 Apr;175(7):1017-1038. doi: 10.1111/bph.14141. Epub 2018 Feb 22.

9.

Protein and lipid interactions - Modulating CFTR trafficking and rescue.

Farinha CM, Miller E, McCarty N.

J Cyst Fibros. 2018 Mar;17(2S):S9-S13. doi: 10.1016/j.jcf.2017.08.014. Epub 2017 Sep 5. Review.

PMID:
28887112
10.

Rab GTPases regulate the trafficking of channels and transporters - a focus on cystic fibrosis.

Farinha CM, Matos P.

Small GTPases. 2018 Mar 4;9(1-2):136-144. doi: 10.1080/21541248.2017.1317700. Epub 2017 May 19. Review.

11.

From the endoplasmic reticulum to the plasma membrane: mechanisms of CFTR folding and trafficking.

Farinha CM, Canato S.

Cell Mol Life Sci. 2017 Jan;74(1):39-55. doi: 10.1007/s00018-016-2387-7. Epub 2016 Oct 3. Review.

PMID:
27699454
12.

Investigating Alternative Transport of Integral Plasma Membrane Proteins from the ER to the Golgi: Lessons from the Cystic Fibrosis Transmembrane Conductance Regulator (CFTR).

Amaral MD, Farinha CM, Matos P, Botelho HM.

Methods Mol Biol. 2016;1459:105-26. doi: 10.1007/978-1-4939-3804-9_7.

PMID:
27665554
13.

EPAC1 activation by cAMP stabilizes CFTR at the membrane by promoting its interaction with NHERF1.

Lobo MJ, Amaral MD, Zaccolo M, Farinha CM.

J Cell Sci. 2016 Jul 1;129(13):2599-612. doi: 10.1242/jcs.185629. Epub 2016 May 20.

14.

Regulatory Crosstalk by Protein Kinases on CFTR Trafficking and Activity.

Farinha CM, Swiatecka-Urban A, Brautigan DL, Jordan P.

Front Chem. 2016 Jan 20;4:1. doi: 10.3389/fchem.2016.00001. eCollection 2016. Review.

15.

Repairing the basic defect in cystic fibrosis - one approach is not enough.

Farinha CM, Matos P.

FEBS J. 2016 Jan;283(2):246-64. doi: 10.1111/febs.13531. Epub 2015 Oct 18. Review.

16.

Increased efficacy of VX-809 in different cellular systems results from an early stabilization effect of F508del-CFTR.

Farinha CM, Sousa M, Canato S, Schmidt A, Uliyakina I, Amaral MD.

Pharmacol Res Perspect. 2015 Aug;3(4):e00152. doi: 10.1002/prp2.152. Epub 2015 Jun 11.

17.

Measurements of Functional Responses in Human Primary Lung Cells as a Basis for Personalized Therapy for Cystic Fibrosis.

Awatade NT, Uliyakina I, Farinha CM, Clarke LA, Mendes K, Solé A, Pastor J, Ramos MM, Amaral MD.

EBioMedicine. 2014 Dec 17;2(2):147-53. doi: 10.1016/j.ebiom.2014.12.005. eCollection 2015.

18.

LMTK2-mediated phosphorylation regulates CFTR endocytosis in human airway epithelial cells.

Luz S, Cihil KM, Brautigan DL, Amaral MD, Farinha CM, Swiatecka-Urban A.

J Biol Chem. 2014 May 23;289(21):15080-93. doi: 10.1074/jbc.M114.563742. Epub 2014 Apr 11.

19.

Revertant mutants modify, but do not rescue, the gating defect of the cystic fibrosis mutant G551D-CFTR.

Xu Z, Pissarra LS, Farinha CM, Liu J, Cai Z, Thibodeau PH, Amaral MD, Sheppard DN.

J Physiol. 2014 May 1;592(9):1931-47. doi: 10.1113/jphysiol.2014.271817. Epub 2014 Mar 3.

20.

Revertants, low temperature, and correctors reveal the mechanism of F508del-CFTR rescue by VX-809 and suggest multiple agents for full correction.

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.

Chem Biol. 2013 Jul 25;20(7):943-55. doi: 10.1016/j.chembiol.2013.06.004.

21.

Control of cystic fibrosis transmembrane conductance regulator membrane trafficking: not just from the endoplasmic reticulum to the Golgi.

Farinha CM, Matos P, Amaral MD.

FEBS J. 2013 Sep;280(18):4396-406. doi: 10.1111/febs.12392. Epub 2013 Jul 5. Review.

22.

Post-translational modifications of CFTR: insight into protein trafficking and cystic fibrosis disease.

Amaral MD, Farinha CM.

FEBS J. 2013 Sep;280(18):4395. doi: 10.1111/febs.12345. Epub 2013 Jul 5. No abstract available.

23.

Rescuing mutant CFTR: a multi-task approach to a better outcome in treating cystic fibrosis.

Amaral MD, Farinha CM.

Curr Pharm Des. 2013;19(19):3497-508. Review.

PMID:
23331027
24.

BAG-1 stabilizes mutant F508del-CFTR in a ubiquitin-like-domain-dependent manner.

Mendes F, Farinha CM, Felício V, Alves PC, Vieira I, Amaral MD.

Cell Physiol Biochem. 2012;30(5):1120-33. doi: 10.1159/000343303. Epub 2012 Oct 5.

25.

CFTR mutations altering CFTR fragmentation.

Tosoni K, Stobbart M, Cassidy DM, Venerando A, Pagano MA, Luz S, Amaral MD, Kunzelmann K, Pinna LA, Farinha CM, Mehta A.

Biochem J. 2013 Jan 1;449(1):295-305. doi: 10.1042/BJ20121240.

26.

Regulation of ENaC biogenesis by the stress response protein SERP1.

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.

Pflugers Arch. 2012 Jun;463(6):819-27. doi: 10.1007/s00424-012-1091-1. Epub 2012 Apr 19.

PMID:
22526458
27.

Contribution of casein kinase 2 and spleen tyrosine kinase to CFTR trafficking and protein kinase A-induced activity.

Luz S, Kongsuphol P, Mendes AI, Romeiras F, Sousa M, Schreiber R, Matos P, Jordan P, Mehta A, Amaral MD, Kunzelmann K, Farinha CM.

Mol Cell Biol. 2011 Nov;31(22):4392-404. doi: 10.1128/MCB.05517-11. Epub 2011 Sep 19.

28.

Antagonistic regulation of cystic fibrosis transmembrane conductance regulator cell surface expression by protein kinases WNK4 and spleen tyrosine kinase.

Mendes AI, Matos P, Moniz S, Luz S, Amaral MD, Farinha CM, Jordan P.

Mol Cell Biol. 2011 Oct;31(19):4076-86. doi: 10.1128/MCB.05152-11. Epub 2011 Aug 1.

29.

Deletion of CFTR translation start site reveals functional isoforms of the protein in CF patients.

Ramalho AS, Lewandowska MA, Farinha CM, Mendes F, Gonçalves J, Barreto C, Harris A, Amaral MD.

Cell Physiol Biochem. 2009;24(5-6):335-46. doi: 10.1159/000257426. Epub 2009 Nov 4.

30.

Solubilizing mutations used to crystallize one CFTR domain attenuate the trafficking and channel defects caused by the major cystic fibrosis mutation.

Pissarra LS, Farinha CM, Xu Z, Schmidt A, Thibodeau PH, Cai Z, Thomas PJ, Sheppard DN, Amaral MD.

Chem Biol. 2008 Jan;15(1):62-9. doi: 10.1016/j.chembiol.2007.11.012.

31.
32.

Characterization of novel airway submucosal gland cell models for cystic fibrosis studies.

da Paula AC, Ramalho AS, Farinha CM, Cheung J, Maurisse R, Gruenert DC, Ousingsawat J, Kunzelmann K, Amaral MD.

Cell Physiol Biochem. 2005;15(6):251-62.

33.
34.

Biochemical methods to assess CFTR expression and membrane localization.

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, Lipecka J, Fritsch J, Gentzsch M, Edelman A, Amaral MD.

J Cyst Fibros. 2004 Aug;3 Suppl 2:73-7.

35.

Antibodies for CFTR studies.

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.

J Cyst Fibros. 2004 Aug;3 Suppl 2:69-72. Review.

36.

Non-PCR methods for the analysis of CFTR transcripts.

Trezise AE, Farinha CM, Heda GD, Harris A, Amaral MD, Mouchel N.

J Cyst Fibros. 2004 Aug;3 Suppl 2:25-8. Review.

37.

Methods for RNA extraction, cDNA preparation and analysis of CFTR transcripts.

Ramalho AS, Beck S, Farinha CM, Clarke LA, Heda GD, Steiner B, Sanz J, Gallati S, Amaral MD, Harris A, Tzetis M.

J Cyst Fibros. 2004 Aug;3 Suppl 2:11-5. Review.

38.

Analysis of genomic CFTR DNA.

Férec C, Le Maréchal C, Audrézet MP, Farinha CM, Amaral MD, Gallati S, Sanz J, Steiner B, Mouchel N, Harris A, Schwarz MJ.

J Cyst Fibros. 2004 Aug;3 Suppl 2:7-10. Review.

39.

A comparison of 14 antibodies for the biochemical detection of the cystic fibrosis transmembrane conductance regulator protein.

Farinha CM, Mendes F, Roxo-Rosa M, Penque D, Amaral MD.

Mol Cell Probes. 2004 Aug;18(4):235-42.

PMID:
15271383
40.

Unusually common cystic fibrosis mutation in Portugal encodes a misprocessed protein.

Mendes F, Roxo Rosa M, Dragomir A, Farinha CM, Roomans GM, Amaral MD, Penque D.

Biochem Biophys Res Commun. 2003 Nov 21;311(3):665-71.

PMID:
14623323
42.

Cystic fibrosis patients with the 3272-26A>G splicing mutation have milder disease than F508del homozygotes: a large European study.

Amaral MD, Pacheco P, Beck S, Farinha CM, Penque D, Nogueira P, Barreto C, Lopes B, Casals T, Dapena J, Gartner S, Vásquez C, Pérez-Frías J, Olveira C, Cabanas R, Estivill X, Tzetis M, Kanavakis E, Doudounakis S, Dörk T, Tümmler B, Girodon-Boulandet E, Cazeneuve C, Goossens M, Blayau M, Verlingue C, Vieira I, Féréc C, Claustres M, des Georges M, Clavel C, Birembaut P, Hubert D, Bienvenu T, Adoun M, Chomel JC, De Boeck K, Cuppens H, Lavinha J.

J Med Genet. 2001 Nov;38(11):777-83. No abstract available.

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