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1

Lung organoids: current uses and future promise.

Barkauskas CE, Chung MI, Fioret B, Gao X, Katsura H, Hogan BL. Barkauskas CE, et al. Development. 2017 Mar 15;144(6):986-997. doi: 10.1242/dev.140103. Development. 2017. PMID: 28292845 Free PMC article. Review.

The proximal and distal regions of the lung contain epithelial cells specialized for different functions: basal, secretory and ciliated cells in the conducting airways and type II and type I cells lining the alveoli. ...We also discuss the potential and limit …

The proximal and distal regions of the lung contain epithelial cells specialized for different functions: basal, secretory and ciliat …

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2

Long-term expanding human airway organoids for disease modeling.

Sachs N, Papaspyropoulos A, Zomer-van Ommen DD, Heo I, Böttinger L, Klay D, Weeber F, Huelsz-Prince G, Iakobachvili N, Amatngalim GD, de Ligt J, van Hoeck A, Proost N, Viveen MC, Lyubimova A, Teeven L, Derakhshan S, Korving J, Begthel H, Dekkers JF, Kumawat K, Ramos E, van Oosterhout MF, Offerhaus GJ, Wiener DJ, Olimpio EP, Dijkstra KK, Smit EF, van der Linden M, Jaksani S, van de Ven M, Jonkers J, Rios AC, Voest EE, van Moorsel CH, van der Ent CK, Cuppen E, van Oudenaarden A, Coenjaerts FE, Meyaard L, Bont LJ, Peters PJ, Tans SJ, van Zon JS, Boj SF, Vries RG, Beekman JM, Clevers H. Sachs N, et al. EMBO J. 2019 Feb 15;38(4):e100300. doi: 10.15252/embj.2018100300. Epub 2019 Jan 14. EMBO J. 2019. PMID: 30643021 Free PMC article.

Organoids are self-organizing 3D structures grown from stem cells that recapitulate essential aspects of organ structure and function. ...Airway organoids derived from cystic fibrosis (CF) patients allow assessment of CFTR function in an organoid swell …

Organoids are self-organizing 3D structures grown from stem cells that recapitulate essential aspects of organ structure and f …

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3

A revised airway epithelial hierarchy includes CFTR-expressing ionocytes.

Montoro DT, Haber AL, Biton M, Vinarsky V, Lin B, Birket SE, Yuan F, Chen S, Leung HM, Villoria J, Rogel N, Burgin G, Tsankov AM, Waghray A, Slyper M, Waldman J, Nguyen L, Dionne D, Rozenblatt-Rosen O, Tata PR, Mou H, Shivaraju M, Bihler H, Mense M, Tearney GJ, Rowe SM, Engelhardt JF, Regev A, Rajagopal J. Montoro DT, et al. Nature. 2018 Aug;560(7718):319-324. doi: 10.1038/s41586-018-0393-7. Epub 2018 Aug 1. Nature. 2018. PMID: 30069044 Free PMC article.

Ionocytes are the major source of transcripts of the cystic fibrosis transmembrane conductance regulator in both mouse (Cftr) and human (CFTR). Knockout of Foxi1 in mouse ionocytes causes loss of Cftr expression and disrupts airway fluid and mucus physiology, phenot …

Ionocytes are the major source of transcripts of the cystic fibrosis transmembrane conductance regulator in both mouse (Cftr) …

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4

The long and winding road: stem cells for cystic fibrosis.

Conese M, Beccia E, Castellani S, Di Gioia S, Colombo C, Angiolillo A, Carbone A. Conese M, et al. Expert Opin Biol Ther. 2018 Mar;18(3):281-292. doi: 10.1080/14712598.2018.1413087. Epub 2017 Dec 8. Expert Opin Biol Ther. 2018. PMID: 29216777 Review.

INTRODUCTION: Cystic fibrosis (CF) is a genetic syndrome with a high mortality rate due to severe lung disease. Despite having several drugs targeting specific mutated CFTR proteins already in clinical trials, new therapies, based on stem cells, are al …

INTRODUCTION: Cystic fibrosis (CF) is a genetic syndrome with a high mortality rate due to severe lung disease. Despite having …

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5

Animal and model systems for studying cystic fibrosis.

Rosen BH, Chanson M, Gawenis LR, Liu J, Sofoluwe A, Zoso A, Engelhardt JF. Rosen BH, et al. J Cyst Fibros. 2018 Mar;17(2S):S28-S34. doi: 10.1016/j.jcf.2017.09.001. Epub 2017 Sep 19. J Cyst Fibros. 2018. PMID: 28939349 Free PMC article. Review.

The cystic fibrosis (CF) field is the beneficiary of five species of animal models that lack functional cystic fibrosis transmembrane conductance regulator (CFTR) channel. ...Gene editing of CFTR mutations in somatic stem cells and induce …

The cystic fibrosis (CF) field is the beneficiary of five species of animal models that lack functional cystic fibro …

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6

Role of the CXCL8-CXCR1/2 Axis in Cancer and Inflammatory Diseases.

Ha H, Debnath B, Neamati N. Ha H, et al. Theranostics. 2017 Apr 7;7(6):1543-1588. doi: 10.7150/thno.15625. eCollection 2017. Theranostics. 2017. PMID: 28529637 Free PMC article. Review.

The CXCL8-CXCR1/2 signaling axis is involved in the pathogenesis of several diseases including chronic obstructive pulmonary diseases (COPD), asthma, cystic fibrosis and cancer. Interaction between CXCL8 secreted by select cancer cells and CXCR1/2 in the tumo …

The CXCL8-CXCR1/2 signaling axis is involved in the pathogenesis of several diseases including chronic obstructive pulmonary diseases (COPD) …

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7

Emerging disease-modifying therapies for sickle cell disease.

Carden MA, Little J. Carden MA, et al. Haematologica. 2019 Sep;104(9):1710-1719. doi: 10.3324/haematol.2018.207357. Epub 2019 Aug 14. Haematologica. 2019. PMID: 31413089 Free PMC article. Review.

To date, sickle cell disease has been cured by hematopoietic stem cell transplantation in approximately 1,000 people, most of whom were children, and significantly ameliorated by gene therapy in a handful of subjects who have only limited follow-up thus far. During a times …

To date, sickle cell disease has been cured by hematopoietic stem cell transplantation in approximately 1,000 people, most of whom we …

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8

Human pluripotent stem cell-derived acinar/ductal organoids generate human pancreas upon orthotopic transplantation and allow disease modelling.

Hohwieler M, Illing A, Hermann PC, Mayer T, Stockmann M, Perkhofer L, Eiseler T, Antony JS, Müller M, Renz S, Kuo CC, Lin Q, Sendler M, Breunig M, Kleiderman SM, Lechel A, Zenker M, Leichsenring M, Rosendahl J, Zenke M, Sainz B Jr, Mayerle J, Costa IG, Seufferlein T, Kormann M, Wagner M, Liebau S, Kleger A. Hohwieler M, et al. Gut. 2017 Mar;66(3):473-486. doi: 10.1136/gutjnl-2016-312423. Epub 2016 Sep 15. Gut. 2017. PMID: 27633923 Free PMC article.

OBJECTIVE: The generation of acinar and ductal cells from human pluripotent stem cells (PSCs) is a poorly studied process, although various diseases arise from this compartment. ...Importantly, cystic fibrosis transmembrane conductance regulator …

OBJECTIVE: The generation of acinar and ductal cells from human pluripotent stem cells (PSCs) is a poorly studied proce …

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9

Directed differentiation of cholangiocytes from human pluripotent stem cells.

Ogawa M, Ogawa S, Bear CE, Ahmadi S, Chin S, Li B, Grompe M, Keller G, Kamath BM, Ghanekar A. Ogawa M, et al. Nat Biotechnol. 2015 Aug;33(8):853-61. doi: 10.1038/nbt.3294. Epub 2015 Jul 13. Nat Biotechnol. 2015. PMID: 26167630

To enable modeling and drug discovery for biliary disease, we describe a protocol that achieves efficient differentiation of biliary epithelial cells (cholangiocytes) from human pluripotent stem cells (hPSCs) through delivery of developmentally relevant cues, …

To enable modeling and drug discovery for biliary disease, we describe a protocol that achieves efficient differentiation of biliary epithel …

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10

Cholangiocytes derived from human induced pluripotent stem cells for disease modeling and drug validation.

Sampaziotis F, de Brito MC, Madrigal P, Bertero A, Saeb-Parsy K, Soares FAC, Schrumpf E, Melum E, Karlsen TH, Bradley JA, Gelson WT, Davies S, Baker A, Kaser A, Alexander GJ, Hannan NRF, Vallier L. Sampaziotis F, et al. Nat Biotechnol. 2015 Aug;33(8):845-852. doi: 10.1038/nbt.3275. Epub 2015 Jul 13. Nat Biotechnol. 2015. PMID: 26167629 Free PMC article.

Here we present an efficient, serum-free protocol for directed differentiation of human induced pluripotent stem cells into cholangiocyte-like cells (CLCs). ...We use CLCs to model in vitro key features of Alagille syndrome, polycystic liver disease and cy …

Here we present an efficient, serum-free protocol for directed differentiation of human induced pluripotent stem cells into ch …

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