Colonic organoids derived from human induced pluripotent stem cells for modeling colorectal cancer and drug testing

With the goal of modeling human disease of the large intestine, we sought to develop an effective protocol for deriving colonic organoids (COs) from differentiated human embryonic stem cells (hESCs) or induced pluripotent stem cells (iPSCs). Extensive gene and immunohistochemical profiling confirmed that the derived COs represent colon rather than small intestine, containing stem cells, transit-amplifying cells, and the expected spectrum of differentiated cells, including goblet and endocrine cells. We applied this strategy to iPSCs derived from patients with familial adenomatous polyposis (FAP-iPSCs) harboring germline mutations in the WNT-signaling-pathway-regulator gene encoding APC, and we generated COs that exhibit enhanced WNT activity and increased epithelial cell proliferation, which we used as a platform for drug testing. Two potential compounds, XAV939 and rapamycin, decreased proliferation in FAP-COs, but also affected cell proliferation in wild-type COs, which thus limits their therapeutic application. By contrast, we found that geneticin, a ribosome-binding antibiotic with translational 'read-through' activity, efficiently targeted abnormal WNT activity and restored normal proliferation specifically in APC-mutant FAP-COs. These studies provide an efficient strategy for deriving human COs, which can be used in disease modeling and drug discovery for colorectal disease.

[1]  G. Fishman,et al.  PCP4 regulates Purkinje cell excitability and cardiac rhythmicity. , 2014, The Journal of clinical investigation.

[2]  Nobutaka Hattori,et al.  Cerebral organoids model human brain development and microcephaly , 2014, Movement disorders : official journal of the Movement Disorder Society.

[3]  L. Pachter,et al.  Human Intestinal Tissue with Adult Stem Cell Properties Derived from Pluripotent Stem Cells , 2014, Stem cell reports.

[4]  S. Orkin,et al.  GATA4 mediates gene repression in the mature mouse small intestine through interactions with friend of GATA (FOG) cofactors. , 2008, Developmental biology.

[5]  D. O'Connor,et al.  Chromogranin A: immunohistology reveals its universal occurrence in normal polypeptide hormone producing endocrine glands. , 1983, Life sciences.

[6]  R. Sasikumar,et al.  Role of Heterozygous APC Mutation in Niche Succession and Initiation of Colorectal Cancer – A Computational Study , 2011, PloS one.

[7]  Toshio Uraoka,et al.  A Colorectal Tumor Organoid Library Demonstrates Progressive Loss of Niche Factor Requirements during Tumorigenesis. , 2016, Cell stem cell.

[8]  V. Smith,et al.  Coordinated expression of 3' hox genes during murine embryonal gut development: an enteric Hox code. , 1999, Gastroenterology.

[9]  Gail H Deutsch,et al.  In vitro generation of human pluripotent stem cell derived lung organoids , 2015, eLife.

[10]  J. Pearson,et al.  The MUC2 gene product: a human intestinal mucin. , 1998, The international journal of biochemistry & cell biology.

[11]  Colin N. Dewey,et al.  RSEM: accurate transcript quantification from RNA-Seq data with or without a reference genome , 2011, BMC Bioinformatics.

[12]  Michael Schumacher,et al.  Modeling human development and disease in pluripotent stem cell-derived gastric organoids , 2014, Nature.

[13]  Takanori Takebe,et al.  Vascularized and functional human liver from an iPSC-derived organ bud transplant , 2013, Nature.

[14]  R. Sherwood,et al.  Wnt signaling specifies and patterns intestinal endoderm , 2011, Mechanisms of Development.

[15]  C. Albanese,et al.  Downregulation of cyclin D1 alters cdk 4- and cdk 2-specific phosphorylation of retinoblastoma protein. , 2000, Molecular cell biology research communications : MCBRC.

[16]  W. W. Nichols,et al.  Species identity of insect cell lines , 1972, In Vitro.

[17]  Michael N. Hall,et al.  mTORC1 mediated translational elongation limits intestinal tumour initiation and growth , 2014, Nature.

[18]  A. Zilberberg,et al.  Restoration of APC gene function in colorectal cancer cells by aminoglycoside- and macrolide-induced read-through of premature termination codons , 2009, Gut.

[19]  Marc W. Kirschner,et al.  Tankyrase inhibition stabilizes axin and antagonizes Wnt signalling , 2009, Nature.

[20]  James D. Johnson,et al.  Reversal of diabetes with insulin-producing cells derived in vitro from human pluripotent stem cells , 2014, Nature Biotechnology.

[21]  C. Schaniel,et al.  Generation of anterior foregut endoderm from human embryonic and induced pluripotent stem cells , 2011, Nature Biotechnology.

[22]  J. Epstein,et al.  Interconversion Between Intestinal Stem Cell Populations in Distinct Niches , 2011, Science.

[23]  Isabelle Duluc,et al.  Neurogenin3 is differentially required for endocrine cell fate specification in the intestinal and gastric epithelium , 2002, The EMBO journal.

[24]  Cole Trapnell,et al.  Ultrafast and memory-efficient alignment of short DNA sequences to the human genome , 2009, Genome Biology.

[25]  M. Taketo Apc gene knockout mice as a model for familial adenomatous polyposis. , 1999, Progress in experimental tumor research.

[26]  R. Moll,et al.  Identification of protein IT of the intestinal cytoskeleton as a novel type I cytokeratin with unusual properties and expression patterns , 1990, The Journal of cell biology.

[27]  Thomas R. Gingeras,et al.  STAR: ultrafast universal RNA-seq aligner , 2013, Bioinform..

[28]  T. Tadakuma,et al.  Carbonic anhydrase I and II as a differentiation marker of human and rat colonic enterocytes , 1998, Research in experimental medicine. Zeitschrift fur die gesamte experimentelle Medizin einschliesslich experimenteller Chirurgie.

[29]  C. Legay,et al.  5-HT metabolism in the intestinal wall of the rat—I. The mucosa , 1983, Neurochemistry International.

[30]  Tony Pawson,et al.  β-Catenin and TCF Mediate Cell Positioning in the Intestinal Epithelium by Controlling the Expression of EphB/EphrinB , 2002, Cell.

[31]  Hans Clevers,et al.  Apc Restoration Promotes Cellular Differentiation and Reestablishes Crypt Homeostasis in Colorectal Cancer , 2015, Cell.

[32]  M. Capecchi,et al.  The intestinal stem cell markers Bmi1 and Lgr5 identify two functionally distinct populations , 2011, Proceedings of the National Academy of Sciences.

[33]  J. Gordon,et al.  Immunocytochemical studies suggest two pathways for enteroendocrine cell differentiation in the colon. , 1992, The American journal of physiology.

[34]  Gordon Keller,et al.  Ductal pancreatic cancer modeling and drug screening using human pluripotent stem cell– and patient-derived tumor organoids , 2015, Nature Medicine.

[35]  M. Bronner,et al.  Gene Signature in Sessile Serrated Polyps Identifies Colon Cancer Subtype , 2016, Cancer Prevention Research.

[36]  P. Scheet,et al.  Genomic Landscape of Colorectal Mucosa and Adenomas , 2016, Cancer Prevention Research.

[37]  Elizabeth E. Hoskins,et al.  Directed differentiation of human pluripotent stem cells into intestinal tissue in vitro , 2010, Nature.

[38]  B. Thiers Induction of Pluripotent Stem Cells from Adult Human Fibroblasts by Defined Factors , 2008 .

[39]  N. Bertin,et al.  A resource of ribosomal RNA-depleted RNA-Seq data from different normal adult and fetal human tissues , 2015, Scientific Data.

[40]  Hayley E. Francies,et al.  Prospective Derivation of a Living Organoid Biobank of Colorectal Cancer Patients , 2015, Cell.

[41]  C. Durno Colonic polyps in children and adolescents. , 2007, Canadian journal of gastroenterology = Journal canadien de gastroenterologie.

[42]  Giusi Moffa,et al.  Wnt secretion is required to maintain high levels of Wnt activity in colon cancer cells , 2013, Nature Communications.

[43]  D. Besselsen,et al.  Combination Chemoprevention of Intestinal Carcinogenesis in a Murine Model of Familial Adenomatous Polyposis , 2008, Nutrition and cancer.

[44]  J. Rousset,et al.  Readthrough of Premature Termination Codons in the Adenomatous Polyposis Coli Gene Restores Its Biological Activity in Human Cancer Cells , 2011, PloS one.

[45]  A. Bretscher,et al.  Villin: the major microfilament-associated protein of the intestinal microvillus. , 1979, Proceedings of the National Academy of Sciences of the United States of America.