Nasal Epithelial Cell-Based Models for Individualized Study in Cystic Fibrosis

Overview

Journal Int J Mol Sci

Publisher MDPI

Specialties Biochemistry
Chemistry
Molecular Biology

Date 2021 Apr 30

PMID 33923202

Citations 11

Authors

Duncan E Keegan

John J Brewington

Affiliations

Soon will be listed here.

Abstract

The emergence of highly effective CFTR modulator therapy has led to significant improvements in health care for most patients with cystic fibrosis (CF). For some, however, these therapies remain inaccessible due to the rarity of their individual variants, or due to a lack of biologic activity of the available therapies for certain variants. One proposed method of addressing this gap is the use of primary human cell-based models, which allow preclinical therapeutic testing and physiologic assessment of relevant tissue at the individual level. Nasal cells represent one such tissue source and have emerged as a powerful model for individual disease study. The ex vivo culture of nasal cells has evolved over time, and modern nasal cell models are beginning to be utilized to predict patient outcomes. This review will discuss both historical and current state-of-the art use of nasal cells for study in CF, with a particular focus on the use of such models to inform personalized patient care.

Citing Articles

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Felipe Montiel A, Fernandez A, Amigo M, Traversi L, Clofent Alarcon D, Reyes K Eur Respir Rev. 2024; 33(174).

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Cystic fibrosis.

Mall M, Burgel P, Castellani C, Davies J, Salathe M, Taylor-Cousar J Nat Rev Dis Primers. 2024; 10(1):53.

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Investigation of CFTR Function in Human Nasal Epithelial Cells Informs Personalized Medicine.

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Comparing Cytology Brushes for Optimal Human Nasal Epithelial Cell Collection: Implications for Airway Disease Diagnosis and Research.

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Revisiting Host-Pathogen Interactions in Cystic Fibrosis Lungs in the Era of CFTR Modulators.

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References

McCarthy C, Brewington J, Harkness B, Clancy J, Trapnell B . Personalised CFTR pharmacotherapeutic response testing and therapy of cystic fibrosis. Eur Respir J. 2018; 51(6). DOI: 10.1183/13993003.02457-2017. View

Veit G, Xu H, Dreano E, Avramescu R, Bagdany M, Beitel L . Structure-guided combination therapy to potently improve the function of mutant CFTRs. Nat Med. 2018; 24(11):1732-1742. PMC: 6301090. DOI: 10.1038/s41591-018-0200-x. View

Laselva O, Bartlett C, Popa A, Ouyang H, Gunawardena T, Gonska T . Emerging preclinical modulators developed for F508del-CFTR have the potential to be effective for ORKAMBI resistant processing mutants. J Cyst Fibros. 2020; 20(1):106-119. DOI: 10.1016/j.jcf.2020.07.015. View

Brewington J, Filbrandt E, LaRosa 3rd F, Ostmann A, Strecker L, Szczesniak R . Detection of CFTR function and modulation in primary human nasal cell spheroids. J Cyst Fibros. 2017; 17(1):26-33. PMC: 5868354. DOI: 10.1016/j.jcf.2017.06.010. View

Roberts N, Al Mubarak R, Francisco D, Kraft M, Chu H . Comparison of paired human nasal and bronchial airway epithelial cell responses to rhinovirus infection and IL-13 treatment. Clin Transl Med. 2018; 7(1):13. PMC: 5931947. DOI: 10.1186/s40169-018-0189-2. View

Whitcutt M, Adler K, Wu R . A biphasic chamber system for maintaining polarity of differentiation of cultured respiratory tract epithelial cells. In Vitro Cell Dev Biol. 1988; 24(5):420-8. DOI: 10.1007/BF02628493. View

Schogler A, Blank F, Brugger M, Beyeler S, Tschanz S, Regamey N . Characterization of pediatric cystic fibrosis airway epithelial cell cultures at the air-liquid interface obtained by non-invasive nasal cytology brush sampling. Respir Res. 2017; 18(1):215. PMC: 5745630. DOI: 10.1186/s12931-017-0706-7. View

Liu Z, Anderson J, Deng L, Mackay S, Bailey J, Kersh L . Human Nasal Epithelial Organoids for Therapeutic Development in Cystic Fibrosis. Genes (Basel). 2020; 11(6). PMC: 7349680. DOI: 10.3390/genes11060603. View

Plotkowski M, Chevillard M, Pierrot D, Altemayer D, Puchelle E . Epithelial respiratory cells from cystic fibrosis patients do not possess specific Pseudomonas aeruginosa-adhesive properties. J Med Microbiol. 1992; 36(2):104-11. DOI: 10.1099/00222615-36-2-104. View

10.

Knowles M, GATZY J, Boucher R . Increased bioelectric potential difference across respiratory epithelia in cystic fibrosis. N Engl J Med. 1981; 305(25):1489-95. DOI: 10.1056/NEJM198112173052502. View

11.

Riordan J, Rommens J, Kerem B, Alon N, Rozmahel R, Grzelczak Z . Identification of the cystic fibrosis gene: cloning and characterization of complementary DNA. Science. 1989; 245(4922):1066-73. DOI: 10.1126/science.2475911. View

12.

Clancy J, Bebok Z, Ruiz F, King C, Jones J, Walker L . Evidence that systemic gentamicin suppresses premature stop mutations in patients with cystic fibrosis. Am J Respir Crit Care Med. 2001; 163(7):1683-92. DOI: 10.1164/ajrccm.163.7.2004001. View

13.

Kunzelmann K, Kathofer S, Greger R . Na+ and Cl- conductances in airway epithelial cells: increased Na+ conductance in cystic fibrosis. Pflugers Arch. 1995; 431(1):1-9. DOI: 10.1007/BF00374371. View

14.

Keating D, Marigowda G, Burr L, Daines C, Mall M, McKone E . VX-445-Tezacaftor-Ivacaftor in Patients with Cystic Fibrosis and One or Two Phe508del Alleles. N Engl J Med. 2018; 379(17):1612-1620. PMC: 6289290. DOI: 10.1056/NEJMoa1807120. View

15.

Neugebauer P, Endepols H, Mickenhagen A, Walger M . Ciliogenesis in submersion and suspension cultures of human nasal epithelial cells. Eur Arch Otorhinolaryngol. 2003; 260(6):325-30. DOI: 10.1007/s00405-002-0562-y. View

16.

de Courcey F, Zholos A, Atherton-Watson H, Williams M, Canning P, Danahay H . Development of primary human nasal epithelial cell cultures for the study of cystic fibrosis pathophysiology. Am J Physiol Cell Physiol. 2012; 303(11):C1173-9. DOI: 10.1152/ajpcell.00384.2011. View

17.

Bridges M, Walker D, Harris R, Wilson B, Davidson A . Cultured human nasal epithelial multicellular spheroids: polar cyst-like model tissues. Biochem Cell Biol. 1991; 69(2-3):102-8. DOI: 10.1139/o91-016. View

18.

Reynolds S, Rios C, Wesolowska-Andersen A, Zhuang Y, Pinter M, Happoldt C . Airway Progenitor Clone Formation Is Enhanced by Y-27632-Dependent Changes in the Transcriptome. Am J Respir Cell Mol Biol. 2016; 55(3):323-36. PMC: 5023026. DOI: 10.1165/rcmb.2015-0274MA. View

19.

Gianotti A, Capurro V, Scudieri P, Galietta L, Moran O, Zegarra-Moran O . Pharmacological rescue of mutant CFTR protein improves the viscoelastic properties of CF mucus. J Cyst Fibros. 2015; 15(3):295-301. DOI: 10.1016/j.jcf.2015.11.003. View

20.

Mosler K, Coraux C, Fragaki K, Zahm J, Bajolet O, Bessaci-Kabouya K . Feasibility of nasal epithelial brushing for the study of airway epithelial functions in CF infants. J Cyst Fibros. 2007; 7(1):44-53. DOI: 10.1016/j.jcf.2007.04.005. View