Publications

Here is a short list of some papers that have used our antibodies:

Bampi GB, Ramalho AS, Santos LA, et al. The Effect of Synonymous Single-Nucleotide Polymorphisms on an Atypical Cystic Fibrosis Clinical Presentation. Life (Basel). 2020; 11(1):14.

Degrugillier F, Aissat A, Prulière-Escabasse V, et al. Phosphorylation of the Chaperone-Like HspB5 Rescues Trafficking and Function of F508del-CFTR. Int J Mol Sci. 2020; 21(14):4844.

Di Fulvio M, Bogdani M, Velasco M, et al. Heterogeneous expression of CFTR in insulin-secreting β-cells of the normal human islet. PLoS One. 2020; 15(12):e0242749.

Gentzsch M, Cholon DM, Quinney NL, et al. Airway Epithelial Inflammation In Vitro Augments the Rescue of Mutant CFTR by Current CFTR Modulator Therapies. Front Pharmacol. 2021; 12:628722.

Gentzsch M, O’Neal WK, Grubb BR, Chen YE, Boucher RC, Sun F Phenotypes of CF rabbits generated by CRISPR/Cas9-mediated disruption of the CFTR gene. JCI Insight Jan 11 2021; 6(1):139813

Gentzsch M, Cholon DM, Quinney NL, et al. Airway Epithelial Inflammation In Vitro Augments the Rescue of Mutant CFTR by Current CFTR Modulator Therapies. Front Pharmacol. 2021; 12:628722.

Guhr Lee TN, Cholon DM, Quinney NL, Gentzsch M, Esther CR Accumulation and Persistence of Ivacaftor in Airway Epithelia with Prolonged Treatment. J Cyst Fibros. 2020 19(5):746-751

He, L, Kennedy A, Houck S, Aleksandrov A, Quinney NL, Gentzsch M, Cholon DM, Randell S, Ren JY, Cyr DM DNAJB12 and Hsp70 Facilitate the Conformation Specific Degradation of Arrested N1303K-CFTR Intermediates by ER Associated-Autophagy. Mol Biol. Cell. 2021; 202: 32(7):538-553

Joynt AT, Evans TA, Pellicore MJ, et al. Evaluation of both exonic and intronic variants for effects on RNA splicing allows for accurate assessment of the effectiveness of precision therapies. PLoS Genet. 2020; 16(10):e1009100

Mancini G, Loberto N, Olioso D, et al. GM1 as Adjuvant of Innovative Therapies for Cystic Fibrosis Disease. Int J Mol Sci. 2020;21(12):4486.

McCravy MS, Quinney NL, Cholon DM, Boyles SE, Jensen TJ, Aleksandrov AA, Donaldson SH, Noone PG, Gentzsch M Personalized medicine for non-classic cystic fibrosis resulting from rare CFTR mutations. Eur Respir J. 2020; 56(1):2000062.

Marquez Loza LI, Cooney AL, Dong Q, et al. Increased CFTR expression and function from an optimized lentiviral vector for cystic fibrosis gene therapy. Mol Ther Methods Clin Dev. 2021; 21:94-106.

Melfi R, Cancemi P, Chiavetta R, Barra V, Lentini L, Di Leonardo A. Investigating REPAIRv2 as a Tool to Edit CFTR mRNA with Premature Stop Codons. Int J Mol Sci. 2020; 21(13):4781.

Michaels WE, Bridges RJ, Hastings ML. Antisense oligonucleotide-mediated correction of CFTR splicing improves chloride secretion in cystic fibrosis patient-derived bronchial epithelial cells. Nucleic Acids Res. 2020; 48(13):7454-7467.

Okuda K, Dang H, Kobayashi Y, Carraro G, et al.  Secretory Cells Dominate Airway CFTR Expression and Function in Human Airway Superficial Epithelia. Am J Respir Crit Care Med. 2020; doi: 10.1164/rccm.202008-3198OC. Online ahead of print. PMID: 33321047

Panigrahi T, D’Souza S, Shetty R, et al. Genistein-Calcitriol Mitigates Hyperosmotic Stress-Induced TonEBP, CFTR Dysfunction, VDR Degradation and Inflammation in Dry Eye Disease. Clin Transl Sci. 2021; 14(1):288-298.

Quaresma MC, Pankonien I, Clarke LA, et al. Mutant CFTR Drives TWIST1 mediated epithelial-mesenchymal transition. Cell Death Dis. 2020; 11(10):920.

Saint-Criq V, Delpiano L, Casement J, Onuora JC, Lin J, Gray MA. Choice of Differentiation Media Significantly Impacts Cell Lineage and Response to CFTR Modulators in Fully Differentiated Primary Cultures of Cystic Fibrosis Human Airway Epithelial Cells. Cells. 2020; 9(9):2137.

Shrestha CL, Zhang S, Wisniewski B, et al. (R)-Roscovitine and CFTR modulators enhance killing of multi-drug resistant Burkholderia cenocepacia by cystic fibrosis macrophages. Sci Rep. 2020; 10(1):21700

Verstegen MMA, Roos FJM, Burka K, et al. Human extrahepatic and intrahepatic cholangiocyte organoids show region-specific differentiation potential and model cystic fibrosis-related bile duct disease. Sci Rep. 2020; 10(1):21900.

Xu J, Livraghi-Butrico A, Hou X, et al. Phenotypes of CF rabbits generated by CRISPR/Cas9-mediated disruption of the CFTR gene. JCI Insight. 2021;6(1):e139813.

Zarei K, Stroik MR, Gansemer ND, et al. Early pathogenesis of cystic fibrosis gallbladder disease in a porcine model. Lab Invest. 2020; 100(11):1388-1399.

Zhang S, Shrestha CL, Wisniewski BL, et al. Consequences of CRISPR-Cas9-Mediated CFTR Knockout in Human Macrophages. Front Immunol. 2020; 11:1871