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Search life-sciences literature (45,103,589 articles, preprints and more)

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Tezacaftor is a direct inhibitor of sphingolipid delta-4 desaturase enzyme (DEGS).
Ciobanu DZ, Liessi N, Tomati V, Capurro V, Bertozzi SM, Summa M, Bertorelli R, Loberto N, Dobi D, Aureli M, Nobbio L, Bandiera T, Pedemonte N, Bassi R, Armirotti A
J Cyst Fibros, S1569-1993(24)00067-5, 23 May 2024
Cited by: 1 article | PMID: 38789319
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Sources of biases in the in vitro testing of nanomaterials: the role of the biomolecular corona.
Castagnola V, Tomati V, Boselli L, Braccia C, Decherchi S, Pompa PP, Pedemonte N, Benfenati F, Armirotti A
Nanoscale Horiz, 9(5):799-816, 29 Apr 2024
Cited by: 0 articles | PMID: 38563642
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SUMOylation Inhibition Enhances Protein Transcription under CMV Promoter: A Lesson from a Study with the F508del-CFTR Mutant.
Borgo C, D'Amore C, Capurro V, Tomati V, Pedemonte N, Bosello Travain V, Salvi M
Int J Mol Sci, 25(4):2302, 15 Feb 2024
Cited by: 0 articles | PMID: 38396982 | PMCID: PMC10889535
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Beyond Kaftrio : mechanistic insights to maximize N1303K-CFTR rescue
Pranke I, Capurro V, Chevalier B, Pesce E, Tomati V, Pastorino C, Hatton A, Urien S, Lena M, Dréano E, Bocciardi R, Zara F, Pantano S, Terlizzi V, Lucanto C, Costa S, Claut L, Daccò V, Poli P, [...] Pedemonte N
bioRxiv, 03 Mar 2024
Cited by: 0 articles | PPR: PPR813860
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De novo variants in DENND5B cause a neurodevelopmental disorder.
Scala M, Tomati V, Ferla M, Lena M, Cohen JS, Fatemi A, Brokamp E, Bican A, Phillips JA, Koziura ME, Nicouleau M, Rio M, Siquier K, Boddaert N, Musante I, Tamburro S, Baldassari S, Iacomino M, Scudieri P, [...] Zara F
Am J Hum Genet, 111(3):529-543, 21 Feb 2024
Cited by: 0 articles | PMID: 38387458 | PMCID: PMC10940048
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Innovative Strategy toward Mutant CFTR Rescue in Cystic Fibrosis: Design and Synthesis of Thiadiazole Inhibitors of the E3 Ligase RNF5.
Brusa I, Sondo E, Pesce E, Tomati V, Gioia D, Falchi F, Balboni B, Ortega Martínez JA, Veronesi M, Romeo E, Margaroli N, Recanatini M, Girotto S, Pedemonte N, Roberti M, Cavalli A
J Med Chem, 66(14):9797-9822, 13 Jul 2023
Cited by: 6 articles | PMID: 37440686 | PMCID: PMC10388311
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Clinical Consequences and Functional Impact of the Rare S737F CFTR Variant and Its Responsiveness to CFTR Modulators.
Terlizzi V, Pesce E, Capurro V, Tomati V, Lena M, Pastorino C, Bocciardi R, Zara F, Centrone C, Taccetti G, Castellani C, Pedemonte N
Int J Mol Sci, 24(7):6576, 31 Mar 2023
Cited by: 4 articles | PMID: 37047546 | PMCID: PMC10095403
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The combination elexacaftor/tezacaftor/ivacaftor (ETI) modulates the de novo synthethic pathway of ceramides in a genotype-independent manner.
Liessi N, Tomati V, Capurro V, Loberto N, Garcia-Aloy M, Franceschi P, Aureli M, Pedemonte N, Armirotti A
J Cyst Fibros, 22(4):680-682, 21 Apr 2023
Cited by: 1 article | PMID: 37088636
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Patient's dermal fibroblasts as disease markers for visceral myopathy.
Viti F, Pramotton FM, Martufi M, Magrassi R, Pedemonte N, Nizzari M, Zanacchi FC, De Michele B, Alampi M, Zambito M, Santamaria G, Bajetto A, Sardar S, Tomati V, Gandullia P, Giampietro C, Florio T, Beltrame F, Vassalli M, Ceccherini I
Biomater Adv, 148:213355, 23 Feb 2023
Cited by: 1 article | PMID: 36893487
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Gain- and Loss-of-Function CFTR Alleles Are Associated with COVID-19 Clinical Outcomes.
Baldassarri M, Zguro K, Tomati V, Pastorino C, Fava F, Croci S, Bruttini M, Picchiotti N, Furini S, Pedemonte N, Gabbi C, Renieri A, Fallerini C, Gen-Covid Multicenter Study
Cells, 11(24):4096, 16 Dec 2022
Cited by: 4 articles | PMID: 36552859 | PMCID: PMC9776607
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Rescue by elexacaftor-tezacaftor-ivacaftor of the G1244E cystic fibrosis mutation's stability and gating defects are dependent on cell background.
Tomati V, Costa S, Capurro V, Pesce E, Pastorino C, Lena M, Sondo E, Di Duca M, Cresta F, Cristadoro S, Zara F, Galietta LJV, Bocciardi R, Castellani C, Lucanto MC, Pedemonte N
J Cyst Fibros, 22(3):525-537, 19 Dec 2022
Cited by: 11 articles | PMID: 36543707
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CFTR Rescue by Lumacaftor (VX-809) Induces an Extensive Reorganization of Mitochondria in the Cystic Fibrosis Bronchial Epithelium.
Braccia C, Christopher JA, Crook OM, Breckels LM, Queiroz RML, Liessi N, Tomati V, Capurro V, Bandiera T, Baldassari S, Pedemonte N, Lilley KS, Armirotti A
Cells, 11(12):1938, 16 Jun 2022
Cited by: 1 article | PMID: 35741067 | PMCID: PMC9222197
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Journey on VX-809-Based Hybrid Derivatives towards Drug-like F508del-CFTR Correctors: From Molecular Modeling to Chemical Synthesis and Biological Assays.
Parodi A, Righetti G, Pesce E, Salis A, Tomati V, Pastorino C, Tasso B, Benvenuti M, Damonte G, Pedemonte N, Cichero E, Millo E
Pharmaceuticals (Basel), 15(3):274, 23 Feb 2022
Cited by: 3 articles | PMID: 35337072 | PMCID: PMC8955485
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The L467F-F508del Complex Allele Hampers Pharmacological Rescue of Mutant CFTR by Elexacaftor/Tezacaftor/Ivacaftor in Cystic Fibrosis Patients: The Value of the Ex Vivo Nasal Epithelial Model to Address Non-Responders to CFTR-Modulating Drugs.
Sondo E, Cresta F, Pastorino C, Tomati V, Capurro V, Pesce E, Lena M, Iacomino M, Baffico AM, Coviello D, Bandiera T, Zara F, Galietta LJV, Bocciardi R, Castellani C, Pedemonte N
Int J Mol Sci, 23(6):3175, 15 Mar 2022
Cited by: 16 articles | PMID: 35328596 | PMCID: PMC8952007
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Targeting the E1 ubiquitin-activating enzyme (UBA1) improves elexacaftor/tezacaftor/ivacaftor efficacy towards F508del and rare misfolded CFTR mutants.
Borgo C, D'Amore C, Capurro V, Tomati V, Sondo E, Cresta F, Castellani C, Pedemonte N, Salvi M
Cell Mol Life Sci, 79(4):192, 16 Mar 2022
Cited by: 10 articles | PMID: 35292885 | PMCID: PMC8924136
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Targeting of Ubiquitin E3 Ligase RNF5 as a Novel Therapeutic Strategy in Neuroectodermal Tumors.
Principi E, Sondo E, Bianchi G, Ravera S, Morini M, Tomati V, Pastorino C, Zara F, Bruno C, Eva A, Pedemonte N, Raffaghello L
Cancers (Basel), 14(7):1802, 01 Apr 2022
Cited by: 4 articles | PMID: 35406574 | PMCID: PMC8997491
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Partial Rescue of F508del-CFTR Stability and Trafficking Defects by Double Corrector Treatment.
Capurro V, Tomati V, Sondo E, Renda M, Borrelli A, Pastorino C, Guidone D, Venturini A, Giraudo A, Mandrup Bertozzi S, Musante I, Bertozzi F, Bandiera T, Zara F, Galietta LJV, Pedemonte N
Int J Mol Sci, 22(10):5262, 17 May 2021
Cited by: 38 articles | PMID: 34067708 | PMCID: PMC8156943
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Discovery of novel VX-809 hybrid derivatives as F508del-CFTR correctors by molecular modeling, chemical synthesis and biological assays.
Parodi A, Righetti G, Pesce E, Salis A, Tasso B, Urbinati C, Tomati V, Damonte G, Rusnati M, Pedemonte N, Cichero E, Millo E
Eur J Med Chem, 208:112833, 12 Sep 2020
Cited by: 7 articles | PMID: 32971410
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Discovery of a picomolar potency pharmacological corrector of the mutant CFTR chloride channel.
Pedemonte N, Bertozzi F, Caci E, Sorana F, Di Fruscia P, Tomati V, Ferrera L, Rodríguez-Gimeno A, Berti F, Pesce E, Sondo E, Gianotti A, Scudieri P, Bandiera T, Galietta LJV
Sci Adv, 6(8):eaay9669, 21 Feb 2020
Cited by: 25 articles | PMID: 32128418 | PMCID: PMC7034990
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Bioactive Thymosin Alpha-1 Does Not Influence F508del-CFTR Maturation and Activity.
Armirotti A, Tomati V, Matthes E, Veit G, Cholon DM, Phuan PW, Braccia C, Guidone D, Gentzsch M, Lukacs GL, Verkman AS, Galietta LJV, Hanrahan JW, Pedemonte N
Sci Rep, 9(1):10310, 16 Jul 2019
Cited by: 7 articles | PMID: 31311979 | PMCID: PMC6635361
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CFTR processing, trafficking and interactions.
Amaral MD, Hutt DM, Tomati V, Botelho HM, Pedemonte N
J Cyst Fibros, 19 Suppl 1:S33-S36, 31 Oct 2019
Cited by: 13 articles | PMID: 31680043
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Thymosin α-1 does not correct F508del-CFTR in cystic fibrosis airway epithelia.
Tomati V, Caci E, Ferrera L, Pesce E, Sondo E, Cholon DM, Quinney NL, Boyles SE, Armirotti A, Ravazzolo R, Galietta LJ, Gentzsch M, Pedemonte N
JCI Insight, 4(7):128935, 04 Apr 2019
Cited by: 1 article | PMID: 30944255 | PMCID: PMC6483642
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Two CFTR mutations within codon 970 differently impact on the chloride channel functionality.
Amato F, Scudieri P, Musante I, Tomati V, Caci E, Comegna M, Maietta S, Manzoni F, Di Lullo AM, De Wachter E, Vanderhelst E, Vanderhelst E, Terlizzi V, Braggion C, Castaldo G, Galietta LJV
Hum Mutat, 40(6):742-748, 28 Mar 2019
Cited by: 26 articles | PMID: 30851139
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The Autophagy Inhibitor Spautin-1 Antagonizes Rescue of Mutant CFTR Through an Autophagy-Independent and USP13-Mediated Mechanism.
Pesce E, Sondo E, Ferrera L, Tomati V, Caci E, Scudieri P, Musante I, Renda M, Baatallah N, Servel N, Hinzpeter A, di Bernardo D, Pedemonte N, Galietta LJV
Front Pharmacol, 9:1464, 13 Dec 2018
Cited by: 14 articles | PMID: 30618756 | PMCID: PMC6300570
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SWATH label-free proteomics for cystic fibrosis research.
Braccia C, Tomati V, Caci E, Pedemonte N, Armirotti A
J Cyst Fibros, 18(4):501-506, 19 Oct 2018
Cited by: 10 articles | PMID: 30348611
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1-25 of 46 results

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Search life-sciences literature (45,103,589 articles, preprints and more)

Search results

Tezacaftor is a direct inhibitor of sphingolipid delta-4 desaturase enzyme (DEGS).

Sources of biases in the in vitro testing of nanomaterials: the role of the biomolecular corona.

SUMOylation Inhibition Enhances Protein Transcription under CMV Promoter: A Lesson from a Study with the F508del-CFTR Mutant.

Beyond Kaftrio : mechanistic insights to maximize N1303K-CFTR rescue

De novo variants in DENND5B cause a neurodevelopmental disorder.

Innovative Strategy toward Mutant CFTR Rescue in Cystic Fibrosis: Design and Synthesis of Thiadiazole Inhibitors of the E3 Ligase RNF5.

Clinical Consequences and Functional Impact of the Rare S737F CFTR Variant and Its Responsiveness to CFTR Modulators.

The combination elexacaftor/tezacaftor/ivacaftor (ETI) modulates the de novo synthethic pathway of ceramides in a genotype-independent manner.

Patient's dermal fibroblasts as disease markers for visceral myopathy.

Gain- and Loss-of-Function CFTR Alleles Are Associated with COVID-19 Clinical Outcomes.

Rescue by elexacaftor-tezacaftor-ivacaftor of the G1244E cystic fibrosis mutation's stability and gating defects are dependent on cell background.

CFTR Rescue by Lumacaftor (VX-809) Induces an Extensive Reorganization of Mitochondria in the Cystic Fibrosis Bronchial Epithelium.

Journey on VX-809-Based Hybrid Derivatives towards Drug-like F508del-CFTR Correctors: From Molecular Modeling to Chemical Synthesis and Biological Assays.

The L467F-F508del Complex Allele Hampers Pharmacological Rescue of Mutant CFTR by Elexacaftor/Tezacaftor/Ivacaftor in Cystic Fibrosis Patients: The Value of the Ex Vivo Nasal Epithelial Model to Address Non-Responders to CFTR-Modulating Drugs.

Targeting the E1 ubiquitin-activating enzyme (UBA1) improves elexacaftor/tezacaftor/ivacaftor efficacy towards F508del and rare misfolded CFTR mutants.

Targeting of Ubiquitin E3 Ligase RNF5 as a Novel Therapeutic Strategy in Neuroectodermal Tumors.

Partial Rescue of F508del-CFTR Stability and Trafficking Defects by Double Corrector Treatment.

Discovery of novel VX-809 hybrid derivatives as F508del-CFTR correctors by molecular modeling, chemical synthesis and biological assays.

Discovery of a picomolar potency pharmacological corrector of the mutant CFTR chloride channel.

Bioactive Thymosin Alpha-1 Does Not Influence F508del-CFTR Maturation and Activity.

CFTR processing, trafficking and interactions.

Thymosin α-1 does not correct F508del-CFTR in cystic fibrosis airway epithelia.

Two CFTR mutations within codon 970 differently impact on the chloride channel functionality.

The Autophagy Inhibitor Spautin-1 Antagonizes Rescue of Mutant CFTR Through an Autophagy-Independent and USP13-Mediated Mechanism.

SWATH label-free proteomics for cystic fibrosis research.

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