{"id":"https://openalex.org/W4282921560","doi":"https://doi.org/10.7554/elife.64430.sa2","title":"Author response: GSK3 inhibition rescues growth and telomere dysfunction in dyskeratosis congenita iPSC-derived type II alveolar epithelial cells","display_name":"Author response: GSK3 inhibition rescues growth and telomere dysfunction in dyskeratosis congenita iPSC-derived type II alveolar epithelial cells","publication_year":2022,"publication_date":"2022-04-04","ids":{"openalex":"https://openalex.org/W4282921560","doi":"https://doi.org/10.7554/elife.64430.sa2"},"language":"en","primary_location":{"id":"doi:10.7554/elife.64430.sa2","doi":"https://doi.org/10.7554/elife.64430.sa2","is_oa":true,"landing_page_url":"https://doi.org/10.7554/elife.64430.sa2","pdf_url":null,"source":{"id":"https://openalex.org/S1336409049","display_name":"eLife","issn_l":"2050-084X","issn":["2050-084X"],"is_oa":true,"is_in_doaj":true,"is_core":true,"listed_in":["anvur-scientific","cwts-core","doaj","dongbi-a","jufo-2","ki-jl-2","medline","norway-2","oarl","poland-200","russia-white-list-1","vabb-shw"],"host_organization":"https://openalex.org/P4310311710","host_organization_name":"eLife Sciences Publications Ltd","host_organization_lineage":["https://openalex.org/P4310311710"],"host_organization_lineage_names":["eLife Sciences Publications Ltd"],"type":"journal"},"license":"cc-by","license_id":"https://openalex.org/licenses/cc-by","version":"publishedVersion","is_accepted":true,"is_published":true,"raw_source_name":null,"raw_type":"peer-review"},"volume":null,"type":"peer-review","indexed_in":["crossref","doaj"],"open_access":{"is_oa":true,"oa_status":"gold","oa_url":"https://doi.org/10.7554/elife.64430.sa2","any_repository_has_fulltext":false},"authorships":[{"author_position":"first","author":{"id":"https://openalex.org/A5091021823","display_name":"Rafael Jesus Fernandez","orcid":"https://orcid.org/0000-0001-9295-4810","observed_orcids":["https://orcid.org/0000-0001-9295-4810"]},"institutions":[{"id":"https://openalex.org/I79576946","display_name":"University of Pennsylvania","ror":"https://ror.org/00b30xv10","country_code":"US","type":"education","lineage":["https://openalex.org/I79576946"]}],"countries":["US"],"is_corresponding":false,"raw_author_name":"Rafael Jesus Fernandez","raw_affiliation_strings":["Cell and Molecular Biology Graduate Group, Perelman School of Medicine, University of Pennsylvania","Medical Scientist Training Program, Perelman School of Medicine, University of Pennsylvania","Perelman School of Medicine, University of Pennsylvania"],"raw_orcid":"https://orcid.org/0000-0001-9295-4810","affiliations":[{"raw_affiliation_string":"Cell and Molecular Biology Graduate Group, Perelman School of Medicine, University of Pennsylvania","institution_ids":["https://openalex.org/I79576946"]},{"raw_affiliation_string":"Medical Scientist Training Program, Perelman School of Medicine, University of Pennsylvania","institution_ids":["https://openalex.org/I79576946"]},{"raw_affiliation_string":"Perelman School of Medicine, University of Pennsylvania","institution_ids":["https://openalex.org/I79576946"]}]},{"author_position":"middle","author":{"id":"https://openalex.org/A5078016749","display_name":"Zachary Gardner","orcid":"https://orcid.org/0000-0003-1905-8686","observed_orcids":["https://orcid.org/0000-0003-1905-8686"]},"institutions":[{"id":"https://openalex.org/I79576946","display_name":"University of Pennsylvania","ror":"https://ror.org/00b30xv10","country_code":"US","type":"education","lineage":["https://openalex.org/I79576946"]}],"countries":["US"],"is_corresponding":false,"raw_author_name":"Zachary JG Gardner","raw_affiliation_strings":["Cell and Molecular Biology Graduate Group, Perelman School of Medicine, University of Pennsylvania","Medical Scientist Training Program, Perelman School of Medicine, University of Pennsylvania","Perelman School of Medicine, University of Pennsylvania"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"Cell and Molecular Biology Graduate Group, Perelman School of Medicine, University of Pennsylvania","institution_ids":["https://openalex.org/I79576946"]},{"raw_affiliation_string":"Medical Scientist Training Program, Perelman School of Medicine, University of Pennsylvania","institution_ids":["https://openalex.org/I79576946"]},{"raw_affiliation_string":"Perelman School of Medicine, University of Pennsylvania","institution_ids":["https://openalex.org/I79576946"]}]},{"author_position":"middle","author":{"id":"https://openalex.org/A5045216788","display_name":"Katherine J. Slovik","orcid":"https://orcid.org/0000-0002-2860-8910","observed_orcids":["https://orcid.org/0000-0002-2860-8910"]},"institutions":[{"id":"https://openalex.org/I79576946","display_name":"University of Pennsylvania","ror":"https://ror.org/00b30xv10","country_code":"US","type":"education","lineage":["https://openalex.org/I79576946"]}],"countries":["US"],"is_corresponding":false,"raw_author_name":"Katherine J Slovik","raw_affiliation_strings":["Institute for Regenerative Medicine, University of Pennsylvania"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"Institute for Regenerative Medicine, University of Pennsylvania","institution_ids":["https://openalex.org/I79576946"]}]},{"author_position":"middle","author":{"id":"https://openalex.org/A5078196069","display_name":"Derek C. Liberti","orcid":"https://orcid.org/0000-0003-2991-9283","observed_orcids":["https://orcid.org/0000-0003-2991-9283"]},"institutions":[{"id":"https://openalex.org/I79576946","display_name":"University of Pennsylvania","ror":"https://ror.org/00b30xv10","country_code":"US","type":"education","lineage":["https://openalex.org/I79576946"]}],"countries":["US"],"is_corresponding":false,"raw_author_name":"Derek C Liberti","raw_affiliation_strings":["Cell and Molecular Biology Graduate Group, Perelman School of Medicine, University of Pennsylvania"],"raw_orcid":"https://orcid.org/0000-0003-2991-9283","affiliations":[{"raw_affiliation_string":"Cell and Molecular Biology Graduate Group, Perelman School of Medicine, University of Pennsylvania","institution_ids":["https://openalex.org/I79576946"]}]},{"author_position":"middle","author":{"id":"https://openalex.org/A5007648506","display_name":"Katrina N. Estep","orcid":"https://orcid.org/0000-0002-8271-8328","observed_orcids":["https://orcid.org/0000-0002-8271-8328"]},"institutions":[{"id":"https://openalex.org/I79576946","display_name":"University of Pennsylvania","ror":"https://ror.org/00b30xv10","country_code":"US","type":"education","lineage":["https://openalex.org/I79576946"]}],"countries":["US"],"is_corresponding":false,"raw_author_name":"Katrina N Estep","raw_affiliation_strings":["Cell and Molecular Biology Graduate Group, Perelman School of Medicine, University of Pennsylvania"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"Cell and Molecular Biology Graduate Group, Perelman School of Medicine, University of Pennsylvania","institution_ids":["https://openalex.org/I79576946"]}]},{"author_position":"middle","author":{"id":"https://openalex.org/A5087046731","display_name":"Wenli Yang","orcid":"https://orcid.org/0000-0002-1873-2336","observed_orcids":["https://orcid.org/0000-0002-1873-2336"]},"institutions":[{"id":"https://openalex.org/I79576946","display_name":"University of Pennsylvania","ror":"https://ror.org/00b30xv10","country_code":"US","type":"education","lineage":["https://openalex.org/I79576946"]}],"countries":["US"],"is_corresponding":false,"raw_author_name":"Wenli Yang","raw_affiliation_strings":["Department of Medicine, Perelman School of Medicine, University of Pennsylvania","Institute for Regenerative Medicine, University of Pennsylvania"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"Department of Medicine, Perelman School of Medicine, University of Pennsylvania","institution_ids":["https://openalex.org/I79576946"]},{"raw_affiliation_string":"Institute for Regenerative Medicine, University of Pennsylvania","institution_ids":["https://openalex.org/I79576946"]}]},{"author_position":"middle","author":{"id":"https://openalex.org/A5101903374","display_name":"Qijun Chen","orcid":"https://orcid.org/0000-0003-0356-7435","observed_orcids":["https://orcid.org/0000-0003-0356-7435"]},"institutions":[{"id":"https://openalex.org/I79576946","display_name":"University of Pennsylvania","ror":"https://ror.org/00b30xv10","country_code":"US","type":"education","lineage":["https://openalex.org/I79576946"]}],"countries":["US"],"is_corresponding":false,"raw_author_name":"Qijun Chen","raw_affiliation_strings":["Department of Pathology and Laboratory Medicine, Perelman School of Medicine, University of Pennsylvania"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"Department of Pathology and Laboratory Medicine, Perelman School of Medicine, University of Pennsylvania","institution_ids":["https://openalex.org/I79576946"]}]},{"author_position":"middle","author":{"id":"https://openalex.org/A5040594512","display_name":"Garrett T. Santini","orcid":"https://orcid.org/0000-0003-0069-7031","observed_orcids":["https://orcid.org/0000-0003-0069-7031"]},"institutions":[{"id":"https://openalex.org/I79576946","display_name":"University of Pennsylvania","ror":"https://ror.org/00b30xv10","country_code":"US","type":"education","lineage":["https://openalex.org/I79576946"]}],"countries":["US"],"is_corresponding":false,"raw_author_name":"Garrett T Santini","raw_affiliation_strings":["Perelman School of Medicine, University of Pennsylvania"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"Perelman School of Medicine, University of Pennsylvania","institution_ids":["https://openalex.org/I79576946"]}]},{"author_position":"middle","author":{"id":"https://openalex.org/A5010929891","display_name":"Javier Vasconcelos Perez","orcid":"https://orcid.org/0000-0002-2899-986X","observed_orcids":["https://orcid.org/0000-0002-2899-986X"]},"institutions":[{"id":"https://openalex.org/I79576946","display_name":"University of Pennsylvania","ror":"https://ror.org/00b30xv10","country_code":"US","type":"education","lineage":["https://openalex.org/I79576946"]}],"countries":["US"],"is_corresponding":false,"raw_author_name":"Javier V Perez","raw_affiliation_strings":["Department of Pathology and Laboratory Medicine, Perelman School of Medicine, University of Pennsylvania"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"Department of Pathology and Laboratory Medicine, Perelman School of Medicine, University of Pennsylvania","institution_ids":["https://openalex.org/I79576946"]}]},{"author_position":"middle","author":{"id":"https://openalex.org/A5068974999","display_name":"Sarah Root","orcid":"https://orcid.org/0000-0001-6845-3020","observed_orcids":["https://orcid.org/0000-0001-6845-3020"]},"institutions":[{"id":"https://openalex.org/I79576946","display_name":"University of Pennsylvania","ror":"https://ror.org/00b30xv10","country_code":"US","type":"education","lineage":["https://openalex.org/I79576946"]}],"countries":["US"],"is_corresponding":false,"raw_author_name":"Sarah Root","raw_affiliation_strings":["College of Arts and Sciences and Vagelos Scholars Program, University of Pennsylvania"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"College of Arts and Sciences and Vagelos Scholars Program, University of Pennsylvania","institution_ids":["https://openalex.org/I79576946"]}]},{"author_position":"middle","author":{"id":"https://openalex.org/A5070300249","display_name":"Ranvir Bhatia","orcid":"https://orcid.org/0000-0002-5226-8776","observed_orcids":["https://orcid.org/0000-0002-5226-8776"]},"institutions":[{"id":"https://openalex.org/I79576946","display_name":"University of Pennsylvania","ror":"https://ror.org/00b30xv10","country_code":"US","type":"education","lineage":["https://openalex.org/I79576946"]}],"countries":["US"],"is_corresponding":false,"raw_author_name":"Ranvir Bhatia","raw_affiliation_strings":["Perelman School of Medicine, University of Pennsylvania"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"Perelman School of Medicine, University of Pennsylvania","institution_ids":["https://openalex.org/I79576946"]}]},{"author_position":"middle","author":{"id":"https://openalex.org/A5011609263","display_name":"John W. Tobias","orcid":"https://orcid.org/0000-0002-5362-7013","observed_orcids":["https://orcid.org/0000-0002-5362-7013"]},"institutions":[{"id":"https://openalex.org/I79576946","display_name":"University of Pennsylvania","ror":"https://ror.org/00b30xv10","country_code":"US","type":"education","lineage":["https://openalex.org/I79576946"]}],"countries":["US"],"is_corresponding":false,"raw_author_name":"John W Tobias","raw_affiliation_strings":["Penn Genomic Analysis Core, Perelman School of Medicine, University of Pennsylvania"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"Penn Genomic Analysis Core, Perelman School of Medicine, University of Pennsylvania","institution_ids":["https://openalex.org/I79576946"]}]},{"author_position":"middle","author":{"id":"https://openalex.org/A5057450814","display_name":"Apoorva Babu","orcid":"https://orcid.org/0000-0003-3661-5261","observed_orcids":["https://orcid.org/0000-0003-3661-5261"]},"institutions":[{"id":"https://openalex.org/I79576946","display_name":"University of Pennsylvania","ror":"https://ror.org/00b30xv10","country_code":"US","type":"education","lineage":["https://openalex.org/I79576946"]}],"countries":["US"],"is_corresponding":false,"raw_author_name":"Apoorva Babu","raw_affiliation_strings":["Penn Cardiovascular Institute, University of Pennsylvania","Penn-CHOP Lung Biology Institute, University of Pennsylvania"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"Penn Cardiovascular Institute, University of Pennsylvania","institution_ids":["https://openalex.org/I79576946"]},{"raw_affiliation_string":"Penn-CHOP Lung Biology Institute, University of Pennsylvania","institution_ids":["https://openalex.org/I79576946"]}]},{"author_position":"middle","author":{"id":"https://openalex.org/A5077653394","display_name":"Michael P. Morley","orcid":"https://orcid.org/0000-0002-0958-7376","observed_orcids":["https://orcid.org/0000-0002-0958-7376"]},"institutions":[{"id":"https://openalex.org/I79576946","display_name":"University of Pennsylvania","ror":"https://ror.org/00b30xv10","country_code":"US","type":"education","lineage":["https://openalex.org/I79576946"]}],"countries":["US"],"is_corresponding":false,"raw_author_name":"Michael P Morley","raw_affiliation_strings":["Penn Cardiovascular Institute, University of Pennsylvania","Penn-CHOP Lung Biology Institute, University of Pennsylvania"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"Penn Cardiovascular Institute, University of Pennsylvania","institution_ids":["https://openalex.org/I79576946"]},{"raw_affiliation_string":"Penn-CHOP Lung Biology Institute, University of Pennsylvania","institution_ids":["https://openalex.org/I79576946"]}]},{"author_position":"middle","author":{"id":"https://openalex.org/A5048588174","display_name":"David B. Frank","orcid":"https://orcid.org/0000-0002-3064-2069","observed_orcids":["https://orcid.org/0000-0002-3064-2069"]},"institutions":[{"id":"https://openalex.org/I1335321130","display_name":"Children's Hospital of Philadelphia","ror":"https://ror.org/01z7r7q48","country_code":"US","type":"funder","lineage":["https://openalex.org/I1335321130"]},{"id":"https://openalex.org/I79576946","display_name":"University of Pennsylvania","ror":"https://ror.org/00b30xv10","country_code":"US","type":"education","lineage":["https://openalex.org/I79576946"]}],"countries":["US"],"is_corresponding":false,"raw_author_name":"David B Frank","raw_affiliation_strings":["Division of Pediatric Cardiology, Department of Pediatrics, Children's Hospital of Philadelphia","Penn-CHOP Lung Biology Institute, University of Pennsylvania"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"Division of Pediatric Cardiology, Department of Pediatrics, Children's Hospital of Philadelphia","institution_ids":["https://openalex.org/I1335321130"]},{"raw_affiliation_string":"Penn-CHOP Lung Biology Institute, University of Pennsylvania","institution_ids":["https://openalex.org/I79576946"]}]},{"author_position":"middle","author":{"id":"https://openalex.org/A5065670232","display_name":"Edward E. Morrisey","orcid":"https://orcid.org/0000-0001-5785-1939","observed_orcids":["https://orcid.org/0000-0001-5785-1939"]},"institutions":[{"id":"https://openalex.org/I79576946","display_name":"University of Pennsylvania","ror":"https://ror.org/00b30xv10","country_code":"US","type":"education","lineage":["https://openalex.org/I79576946"]}],"countries":["US"],"is_corresponding":false,"raw_author_name":"Edward E Morrisey","raw_affiliation_strings":["Institute for Regenerative Medicine, University of Pennsylvania","Penn Cardiovascular Institute, University of Pennsylvania","Penn-CHOP Lung Biology Institute, University of Pennsylvania"],"raw_orcid":"https://orcid.org/0000-0001-5785-1939","affiliations":[{"raw_affiliation_string":"Institute for Regenerative Medicine, University of Pennsylvania","institution_ids":["https://openalex.org/I79576946"]},{"raw_affiliation_string":"Penn Cardiovascular Institute, University of Pennsylvania","institution_ids":["https://openalex.org/I79576946"]},{"raw_affiliation_string":"Penn-CHOP Lung Biology Institute, University of Pennsylvania","institution_ids":["https://openalex.org/I79576946"]}]},{"author_position":"middle","author":{"id":"https://openalex.org/A5003098266","display_name":"Christopher J. Lengner","orcid":"https://orcid.org/0000-0002-0574-5189","observed_orcids":["https://orcid.org/0000-0002-0574-5189","https://orcid.org/0000-0002-5406-8997"]},"institutions":[{"id":"https://openalex.org/I79576946","display_name":"University of Pennsylvania","ror":"https://ror.org/00b30xv10","country_code":"US","type":"education","lineage":["https://openalex.org/I79576946"]}],"countries":["US"],"is_corresponding":false,"raw_author_name":"Christopher J Lengner","raw_affiliation_strings":["Department of Biomedical Sciences, School of Veterinary Medicine, University of Pennsylvania","Institute for Regenerative Medicine, University of Pennsylvania"],"raw_orcid":"https://orcid.org/0000-0002-0574-5189","affiliations":[{"raw_affiliation_string":"Department of Biomedical Sciences, School of Veterinary Medicine, University of Pennsylvania","institution_ids":["https://openalex.org/I79576946"]},{"raw_affiliation_string":"Institute for Regenerative Medicine, University of Pennsylvania","institution_ids":["https://openalex.org/I79576946"]}]},{"author_position":"last","author":{"id":"https://openalex.org/A5103054205","display_name":"F Brad Johnson","orcid":"https://orcid.org/0000-0002-7443-7227","observed_orcids":["https://orcid.org/0000-0002-7443-7227"]},"institutions":[{"id":"https://openalex.org/I79576946","display_name":"University of Pennsylvania","ror":"https://ror.org/00b30xv10","country_code":"US","type":"education","lineage":["https://openalex.org/I79576946"]}],"countries":["US"],"is_corresponding":false,"raw_author_name":"F Brad Johnson","raw_affiliation_strings":["Department of Pathology and Laboratory Medicine, Perelman School of Medicine, University of Pennsylvania","Institute for Regenerative Medicine, University of Pennsylvania","Institute on Aging, University of Pennsylvania"],"raw_orcid":"https://orcid.org/0000-0002-7443-7227","affiliations":[{"raw_affiliation_string":"Department of Pathology and Laboratory Medicine, Perelman School of Medicine, University of Pennsylvania","institution_ids":["https://openalex.org/I79576946"]},{"raw_affiliation_string":"Institute for Regenerative Medicine, University of Pennsylvania","institution_ids":["https://openalex.org/I79576946"]},{"raw_affiliation_string":"Institute on Aging, University of Pennsylvania","institution_ids":["https://openalex.org/I79576946"]}]}],"institutions":[],"countries_distinct_count":1,"institutions_distinct_count":2,"corresponding_author_ids":[],"corresponding_institution_ids":[],"apc_list":{"value":3000,"currency":"USD","value_usd":2500},"apc_paid":{"value":3000,"currency":"USD","value_usd":2500},"fwci":null,"has_fulltext":false,"cited_by_count":0,"citation_normalized_percentile":null,"cited_by_percentile_year":null,"biblio":{"volume":null,"issue":null,"first_page":null,"last_page":null},"is_retracted":false,"is_paratext":false,"is_xpac":false,"primary_topic":{"id":"https://openalex.org/T10937","display_name":"Telomeres, Telomerase, and Senescence","score":0.9932000041007996,"subfield":{"id":"https://openalex.org/subfields/2737","display_name":"Physiology"},"field":{"id":"https://openalex.org/fields/27","display_name":"Medicine"},"domain":{"id":"https://openalex.org/domains/4","display_name":"Health Sciences"}},"topics":[{"id":"https://openalex.org/T10937","display_name":"Telomeres, Telomerase, and Senescence","score":0.9932000041007996,"subfield":{"id":"https://openalex.org/subfields/2737","display_name":"Physiology"},"field":{"id":"https://openalex.org/fields/27","display_name":"Medicine"},"domain":{"id":"https://openalex.org/domains/4","display_name":"Health Sciences"}},{"id":"https://openalex.org/T10505","display_name":"Pluripotent Stem Cells Research","score":0.0017000000225380063,"subfield":{"id":"https://openalex.org/subfields/1312","display_name":"Molecular Biology"},"field":{"id":"https://openalex.org/fields/13","display_name":"Biochemistry, Genetics and Molecular Biology"},"domain":{"id":"https://openalex.org/domains/1","display_name":"Life Sciences"}},{"id":"https://openalex.org/T10870","display_name":"Interstitial Lung Diseases and Idiopathic Pulmonary Fibrosis","score":0.0010999999940395355,"subfield":{"id":"https://openalex.org/subfields/2740","display_name":"Pulmonary and Respiratory Medicine"},"field":{"id":"https://openalex.org/fields/27","display_name":"Medicine"},"domain":{"id":"https://openalex.org/domains/4","display_name":"Health Sciences"}}],"keywords":[{"id":"https://openalex.org/keywords/gsk-3-inhibition","display_name":"GSK3 inhibition","score":0.9810000061988831},{"id":"https://openalex.org/keywords/telomere-dysfunction","display_name":"telomere dysfunction","score":0.9480000138282776},{"id":"https://openalex.org/keywords/dyskeratosis-congenita","display_name":"dyskeratosis congenita","score":0.9909999966621399},{"id":"https://openalex.org/keywords/alveolar-type-ii-cells","display_name":"alveolar type II cells","score":0.9670000076293945},{"id":"https://openalex.org/keywords/induced-pluripotent-stem-cells","display_name":"induced pluripotent stem cells","score":0.9300000071525574},{"id":"https://openalex.org/keywords/cellular-senescence","display_name":"cellular senescence","score":0.984000027179718},{"id":"https://openalex.org/keywords/wnt-signaling","display_name":"Wnt signaling","score":0.9850000143051147},{"id":"https://openalex.org/keywords/pulmonary-fibrosis","display_name":"pulmonary fibrosis","score":0.796999990940094},{"id":"https://openalex.org/keywords/gene-expression-changes","display_name":"gene expression changes","score":0.5609999895095825}],"concepts":[{"id":"https://openalex.org/C2779285011","wikidata":"https://www.wikidata.org/wiki/Q3709312","display_name":"Dyskeratosis congenita","level":4,"score":0.9515852928161621},{"id":"https://openalex.org/C177336024","wikidata":"https://www.wikidata.org/wiki/Q14872779","display_name":"Telomere","level":3,"score":0.9181013703346252},{"id":"https://openalex.org/C94581717","wikidata":"https://www.wikidata.org/wiki/Q331839","display_name":"Telomerase","level":3,"score":0.8557286262512207},{"id":"https://openalex.org/C137620995","wikidata":"https://www.wikidata.org/wiki/Q155769","display_name":"Wnt signaling pathway","level":3,"score":0.8484964370727539},{"id":"https://openalex.org/C86803240","wikidata":"https://www.wikidata.org/wiki/Q420","display_name":"Biology","level":0,"score":0.49726155400276184},{"id":"https://openalex.org/C502942594","wikidata":"https://www.wikidata.org/wiki/Q3421914","display_name":"Cancer research","level":1,"score":0.45587509870529175},{"id":"https://openalex.org/C95444343","wikidata":"https://www.wikidata.org/wiki/Q7141","display_name":"Cell biology","level":1,"score":0.43587034940719604},{"id":"https://openalex.org/C107459253","wikidata":"https://www.wikidata.org/wiki/Q674925","display_name":"Induced pluripotent stem cell","level":4,"score":0.4273376762866974},{"id":"https://openalex.org/C54355233","wikidata":"https://www.wikidata.org/wiki/Q7162","display_name":"Genetics","level":1,"score":0.3056384325027466},{"id":"https://openalex.org/C62478195","wikidata":"https://www.wikidata.org/wiki/Q828130","display_name":"Signal transduction","level":2,"score":0.2760622799396515},{"id":"https://openalex.org/C104317684","wikidata":"https://www.wikidata.org/wiki/Q7187","display_name":"Gene","level":2,"score":0.08570218086242676},{"id":"https://openalex.org/C145103041","wikidata":"https://www.wikidata.org/wiki/Q1151519","display_name":"Embryonic stem cell","level":3,"score":0.05829980969429016}],"mesh":[],"locations_count":1,"locations":[{"id":"doi:10.7554/elife.64430.sa2","doi":"https://doi.org/10.7554/elife.64430.sa2","is_oa":true,"landing_page_url":"https://doi.org/10.7554/elife.64430.sa2","pdf_url":null,"source":{"id":"https://openalex.org/S1336409049","display_name":"eLife","issn_l":"2050-084X","issn":["2050-084X"],"is_oa":true,"is_in_doaj":true,"is_core":true,"listed_in":["anvur-scientific","cwts-core","doaj","dongbi-a","jufo-2","ki-jl-2","medline","norway-2","oarl","poland-200","russia-white-list-1","vabb-shw"],"host_organization":"https://openalex.org/P4310311710","host_organization_name":"eLife Sciences Publications Ltd","host_organization_lineage":["https://openalex.org/P4310311710"],"host_organization_lineage_names":["eLife Sciences Publications Ltd"],"type":"journal"},"license":"cc-by","license_id":"https://openalex.org/licenses/cc-by","version":"publishedVersion","is_accepted":true,"is_published":true,"raw_source_name":null,"raw_type":"peer-review"}],"best_oa_location":{"id":"doi:10.7554/elife.64430.sa2","doi":"https://doi.org/10.7554/elife.64430.sa2","is_oa":true,"landing_page_url":"https://doi.org/10.7554/elife.64430.sa2","pdf_url":null,"source":{"id":"https://openalex.org/S1336409049","display_name":"eLife","issn_l":"2050-084X","issn":["2050-084X"],"is_oa":true,"is_in_doaj":true,"is_core":true,"listed_in":["anvur-scientific","cwts-core","doaj","dongbi-a","jufo-2","ki-jl-2","medline","norway-2","oarl","poland-200","russia-white-list-1","vabb-shw"],"host_organization":"https://openalex.org/P4310311710","host_organization_name":"eLife Sciences Publications Ltd","host_organization_lineage":["https://openalex.org/P4310311710"],"host_organization_lineage_names":["eLife Sciences Publications Ltd"],"type":"journal"},"license":"cc-by","license_id":"https://openalex.org/licenses/cc-by","version":"publishedVersion","is_accepted":true,"is_published":true,"raw_source_name":null,"raw_type":"peer-review"},"sustainable_development_goals":[{"id":"https://metadata.un.org/sdg/3","score":0.9537000060081482,"display_name":"Good health and well-being"}],"study_designs":[],"awards":[],"funders":[],"has_content":{"grobid_xml":false,"pdf":false},"content_urls":null,"referenced_works_count":0,"referenced_works":[],"related_works":["https://openalex.org/W4280520910","https://openalex.org/W2511441887","https://openalex.org/W2936437338","https://openalex.org/W7161556168","https://openalex.org/W1967991223","https://openalex.org/W2526978145","https://openalex.org/W3204499545","https://openalex.org/W4404102492","https://openalex.org/W4220984151","https://openalex.org/W4214854173"],"abstract_inverted_index":{"Article":[0,24],"Figures":[1],"and":[2,11,25,49,87,117,128,149,192,204,214,224,231,233,246,279,281,292,350,364,373,380,393,436,460,559,565,611,668,712,734,782,803,896,907,993,1000,1005,1045,1071,1156,1173,1184,1204,1281,1299,1354,1388,1476,1505,1531,1611,1626,1640,1652,1662,1689,1842,1966,1993,2009,2041,2067,2163,2180,2240,2248,2257,2273,2315,2355,2386,2683,2688,2750,2787,2827,2860,2870,2897,2967,3029,3045,3050,3084,3095,3116,3180,3213,3227,3307,3309,3337,3372,3410,3504,3543,3920,3952,4136,4170,4175,4187,4198,4213,4233,4246,4282,4340,4342,4368,4374,4378,4399],"data":[3,227,1514,1727,2442,2467,2470,3102,3610,3654,3702,3755,3758,4007],"Abstract":[4,29],"Editor's":[5,167],"evaluation":[6,168],"Introduction":[7,258],"Results":[8,1216],"Discussion":[9,4105],"Materials":[10],"methods":[12],"Appendix":[13,15],"1":[14,1552,1629,1728,2468,2760,2826,3134,3556,3756],"2":[16,2196,2198,2792,2828,3618,3662,3710],"Data":[17,1730],"availability":[18],"References":[19],"Decision":[20,250],"letter":[21,251],"Author":[22],"response":[23,509,914,2733,3008],"author":[26],"information":[27],"Metrics":[28],"Dyskeratosis":[30,178,244,259,1562,2206],"congenita":[31,245,260,1563,2207],"(DC)":[32,261,3510],"is":[33,62,221,262,341,348,383,572,690,702,868,930,973,991,1020,1830,1875,2107,2267,2562,2595,3110,4050,4055,4152,4231,4298],"a":[34,172,207,263,330,342,425,505,594,646,726,1218,1234,1282,1442,1452,1831,1876,2126,2151,2258,2268,2278,2302,2342,2370,2402,2428,2691,2744,2762,2799,2892,2951,2986,2993,3156,3279,3328,3402,3429,3600,3646,3690,3960,4142,4204,4355],"rare":[35,264],"genetic":[36,265,420],"disorder":[37,266],"characterized":[38,182,267,1455,4153],"by":[39,185,268,289,730,788,825,1095,1407,1456,1470,1479,1544,1710,2036,2330,2401,2714,2780,2956,2964,2977,3082,3113,3398,3730,4047,4147,4154,4259],"deficiencies":[40,297],"in":[41,120,131,195,201,298,305,315,410,432,481,514,577,581,599,604,615,620,670,707,842,848,864,913,946,975,1011,1053,1064,1133,1139,1151,1199,1213,1239,1342,1360,1390,1441,1502,1854,1878,1989,2000,2015,2032,2055,2075,2085,2090,2251,2271,2281,2463,2568,2601,2638,2652,2776,2802,2895,2918,2931,2936,2953,3007,3054,3107,3160,3287,3341,3375,3428,3507,3743,3910,3923,3963,3971,3988,4000,4096,4144,4223,4308,4338,4407],"telomere":[42,247,299,575,597,666,697,732,763,786,793,846,1073,1108,1154,1185,1205,1343,1524,2062,2077,2083,2098,2146,2376,2425,2461,2531,2621,3033,3063,3067,3370,3505,3694,3822,3934,3965,4025,4058,4097,4125,4196,4227,4373],"maintenance":[43,794],"leading":[44],"to":[45,64,100,189,241,303,427,504,511,535,700,744,852,915,943,981,1022,1032,1060,1083,1105,1371,1385,1445,1522,1576,1586,1743,1748,1927,2193,2645,3009,3062,3079,3166,3390,3442,3526,3771,3776,3994,3997,4057,4077,4081,4113,4122,4208,4372],"very":[46,2674],"short":[47,103,560,643,1382,2114,2129,2179,2431],"telomeres":[48,104,116,294,437,561,614,644,1163,1355,1383,2115,2130,2155,2157,2182,2414,2432,2445,3011,3115,3977,4037,4121,4138],"the":[50,121,140,151,219,226,234,306,317,353,377,407,416,492,523,578,746,760,770,797,806,821,832,910,926,963,967,1001,1033,1043,1046,1077,1134,1190,1201,1226,1256,1303,1306,1333,1361,1404,1431,1434,1491,1494,1517,1528,1533,1545,1588,1591,1658,1663,1672,1711,1767,1770,1795,1802,1816,1843,1859,1902,1925,1944,1957,1964,2003,2111,2178,2223,2331,2435,2447,2457,2492,2550,2554,2563,2583,2587,2596,2624,2653,2704,2726,2730,2740,2794,2830,2835,3004,3023,3039,3184,3214,3355,3392,3421,3448,3469,3484,3731,3933,3972,4015,4083,4101,4166,4224,4289,4332,4335,4343,4358,4388,4396],"premature":[51],"onset":[52],"of":[53,71,123,126,142,158,238,276,308,321,344,406,418,422,441,496,522,609,648,721,762,773,792,800,805,823,839,862,909,1008,1016,1028,1037,1042,1049,1081,1098,1197,1207,1274,1297,1305,1433,1473,1493,1536,1590,1607,1637,1657,1665,1671,1694,1739,1745,1769,1789,1797,1813,1835,1845,1861,1895,1943,1948,1980,2002,2024,2045,2113,2128,2145,2153,2187,2222,2229,2235,2253,2255,2305,2345,2373,2408,2420,2430,2460,2481,2506,2518,2530,2546,2579,2616,2665,2694,2725,2746,2806,2819,2883,2945,2973,2989,2991,3003,3022,3038,3047,3092,3143,3186,3209,3225,3253,3281,3301,3311,3330,3357,3378,3425,3468,3486,3532,3538,3552,3564,3573,3603,3649,3693,3767,3773,3789,3805,3821,3839,3846,3867,3870,3881,3884,3897,3900,3907,3936,3974,4017,4031,4070,4226,4273,4299,4306,4334,4346,4357,4394,4404],"certain":[54,277],"age-related":[55],"diseases,":[56],"including":[57,796,2642,3299,4165],"pulmonary":[58,282,3233],"fibrosis":[59,283,3234],"(PF).":[60,284],"PF":[61,328,340,411,428,537,606,649,675,701,949,984,1012,1031,1054,1084,1212,2811,2820,4164,4211,4217,4309,4408],"thought":[63],"derive":[65],"from":[66,296,1024,1229,1781,1811,1889,1936,1984,2479,2611,3451,3616,3660,3708],"epithelial":[67,75,434,489,872,1572,1778,2215,2489,3152,3518,3851],"failure,":[68,271],"particularly":[69,324,580],"that":[70,347,430,484,512,642,665,812,834,932,1125,1145,1192,1329,1349,1438,1516,1873,1901,2121,2158,2177,2183,2703,2995,3027,3104,3117,3366,3480,3859,3889,4009,4034,4038,4043,4053,4134,4158,4261],"type":[72,486,1569,1610,1775,2212,2247,2486,3149,3515,3541,3848,4401],"II":[73,487,1570,1776,2213,2487,3150,3516,3849],"alveolar":[74,356,433,488,1571,1777,2214,2488,3151,3517,3850],"(AT2)":[76,490],"cells,":[77,491,977,2265,2641,2656,3058,3951,4020],"which":[78,138,526,731,819,1448,2106,2889,3059,3406,3464],"are":[79,229,287,369,438,562,568,645,979,1126,1706,2711,2934,3035,3339,3588,3615,3634,3659,3678,3707,4039,4139,4363],"highly":[80,1832],"dependent":[81,995,3158,3431,4243,4295],"on":[82,253,414,1595,1643],"Wnt":[83,132,144,159,202,208,736,774,813,835,858,866,969,989,1009,1171,1193,2646,2885,2901,2914,2962,2978,3005,3041,3105,3119,3161,3211,3316,3413,4185,4229,4274,4296,4347,4405],"signaling":[84,737,775,867,990,1010,2647,2752,2853,2886,2963,3106,3120,3127,3212,4230,4245,4275,4297,4406],"during":[85,4072],"development":[86,874],"adult":[88,911],"regeneration.":[89],"We":[90,1089,1269,1347,1392,2672,2797,3457,3927,4106,4132],"use":[91],"human":[92,637,713,1072,1099,1261,4109,4391],"induced":[93,714,983,1564,1771,2148,2208,2378,2482,2533,3145,3511,3696,3824],"pluripotent":[94,715,1565,1772,2209,2483,3146,3512],"stem":[95,494,716,748,778,1202,1566,1773,2210,2484,3147,3513],"cell-derived":[96,718,1774,2211,2485,3148,3514],"AT2":[97,106,582,602,671,695,849,976,1092,1102,1208,1227,1703,1821,1836,1879,1897,1958,3108,4129,4220],"(iAT2)":[98,3153,3519,3852],"cells":[99,112,435,495,583,603,672,850,1148,1159,1209,1223,1228,1331,1380,1402,1573,1661,1667,1674,1683,1698,1746,1779,1799,1837,1848,1863,1888,1915,1934,1938,1983,2046,2070,2124,2190,2216,2237,2300,2306,2324,2346,2374,2395,2437,2490,2509,2521,2536,2685,2805,2921,2939,3088,3109,3154,3169,3205,3277,3326,3345,3427,3472,3548,3598,3604,3644,3688,3724,3745,3774,3792,3808,3827,3853,3872,3942,3945,3955,3990,4002,4118,4157,4181],"model":[101,176,184,1225,4207],"how":[102,694,845,1107,3168,3183,3528,3930,4124,4214,4263],"affect":[105,1907,4128,4219],"cells.":[107,1597,2669,3652,3700,4149],"Cultured":[108],"DC":[109,322,530,722,1214,1230,1237,1913,1932,2122,2138,2188,2639,2654,2803,2919,2937,3056,3086,3358,3379,3393,3487,3533,3924,3937,4018,4115,4178],"mutant":[110,1146,1279,1614,1856,1967,2016,2026,2298,2339,2367,2684,3470,3546,3596,3642,3686,3989,4116,4179],"iAT2":[111,1147,1158,1379,1401,1596,1616,1673,1678,1697,1890,1918,1933,1968,1985,2027,2069,2123,2139,2189,2236,2299,2411,2423,2436,2640,2655,2668,2804,2920,2938,3057,3087,3204,3276,3325,3344,3359,3394,3426,3471,3488,3597,3643,3687,3744,3871,3886,3925,3941,3982,4019,4117,4148,4156,4180],"accumulate":[113,1680,2184],"shortened,":[114],"uncapped":[115,293,1162,2154,2181,2715,3010,4137],"manifest":[118],"defects":[119,130,194,200,213,304,431,724,1206,1501,1518,2775,3374,3506],"growth":[122,1183,1453,1504,3356,3423,3449,3467,3485,3503,3531,4016,4084],"alveolospheres,":[124],"hallmarks":[125,608,1947,2228],"senescence,":[127],"apparent":[129,1469,3969],"signaling.":[133,3162,4177,4250],"The":[134,180,1466,3018],"GSK3":[135,817,824,1176,3352,3367,3404,3461,3481,4010,4191],"inhibitor,":[136,2261,3405,3462],"CHIR99021,":[137,1180,3401,3445,3593,3639,3683],"mimics":[139],"output":[141],"canonical":[143,735,968,2913,3040,3125,3412,4184],"signaling,":[145,203,1172,2749,2915,4174,4186,4348],"enhances":[146],"telomerase":[147,216,1339,1350,1537,3741,3984,4032,4054,4103,4194],"activity":[148,1340,1351,3742,3985,4033],"rescues":[150,1181,3502,4014,4024,4193],"defects.":[152],"These":[153,285,809,1187,1513,2174,2833,3075,3101,3477,4251,4285,4385],"findings":[154,1188,2175,3478],"support":[155,738],"further":[156,1962,2649],"investigation":[157],"agonists":[160,815,1194],"as":[161,1179,1461,1463,1622,1752,1754,1794,1995,1997,2275,2277,2328,2434,2566,2599,2772,2774,4257,4267],"potential":[162,1034,1541,4215],"therapies":[163,368],"for":[164,177,596,870,937,1129,1414,1422,1624,1631,1731,2110,2348,2471,2558,2591,2761,2793,2816,2829,2912,3172,3558,3611,3655,3703,3740,3759,4390],"DC-related":[165],"pathologies.":[166],"This":[169,2561,2594,2877,4150,4201],"study":[170,220,4411],"reports":[171,2456,4291],"new":[173,4205,4216],"cell":[174,696,749,779,873,1051,1093,1203,1263,1275,1367,1398,1567,1704,1880,1891,1919,1959,2004,2164,2259,2424,3216,3520,3983,4111,4130,4221,4400],"line":[175,1264,1280,1287,1368],"congenita.":[179],"authors":[181],"this":[183,765,3446],"examining":[186],"its":[187,193,1372],"ability":[188],"form":[190,1386,1577],"organoids":[191,1094],"telomeres.":[196,2716],"Transcriptomics":[197],"analysis":[198,2615,2767,2878,3222,3249,3293],"unveiled":[199],"treatment":[205,1174,3399,3569,3794,3810,3829],"with":[206,358,424,574,593,777,948,1076,1153,1160,1169,1175,1211,1332,1345,1357,1381,1498,1553,1579,1669,1758,1786,1929,2079,2136,2185,2197,2307,2325,2375,2396,2416,2511,2523,2538,2627,2754,2810,2891,2928,2970,3133,3232,3283,3304,3314,3332,3400,3444,3492,3549,3570,3590,3605,3636,3680,3725,3781,3795,3798,3811,3814,3830,3833,3855,3874,3946,3992,4091,4100,4119,4141,4163,4189,4370],"agonist":[209],"could":[210,837,4035],"rescue":[211,828,3354,3391],"these":[212,566,829,1395,1922,2010,4006,4349],"enhance":[215],"activity.":[217,4104],"Overall,":[218],"well":[222,1462,1753,1996,2276,2773],"designed":[223],"executed,":[225],"presented":[228],"clear":[230,4364],"convincing":[232],"conclusions":[235],"should":[236],"be":[237,838,861,944,1196,3080,3122,3129,4045,4235,4255],"great":[239],"interest":[240],"researchers":[242],"studying":[243],"biology.":[248],"https://doi.org/10.7554/eLife.64430.sa0":[249],"Reviews":[252],"Sciety":[254],"eLife's":[255],"review":[256],"process":[257],"bone":[269,318],"marrow":[270,319],"skin":[272],"abnormalities,":[273],"elevated":[274,3123],"risk":[275,563],"cancers,":[278],"liver":[280],"pathologies":[286],"caused":[288,1543],"abnormally":[290],"shortened":[291,1356,3114,4120,4135],"arising":[295],"maintenance,":[300],"typically":[301],"due":[302,1521],"action":[307],"telomerase.":[309],"Significant":[310],"progress":[311],"has":[312],"been":[313,533,941],"made":[314],"treating":[316],"failure":[320],"patients,":[323,1231],"via":[325,4060],"transplantation,":[326],"but":[327,1447,2052,2690,3967,4021],"remains":[329,1013],"major":[331,4240],"life-limiting":[332],"pathology":[333],"(Agarwal,":[334],"2018;":[335,395,902,920],"Dietz":[336],"et":[337,389,397,401,444,448,452,456,464,468,472,476,499,517,539,543,547,551,585,589,624,628,632,651,658,677,681,685,752,756,876,880,884,888,892,900,904,918,922,951,955,959,986,997,1115,1119,1244,1248,1252,1266,1317,1321,1325,1409,1839,2103,2169,2838,2842,2849,2855,2863,2867,2874,3015,3072,3381,3385,4064,4278,4302,4311,4315,4319,4323,4327,4382],"al.,":[338,390,398,402,445,449,453,457,465,469,473,477,500,518,540,544,548,552,586,590,625,629,633,652,659,678,682,686,753,757,877,881,885,889,893,901,905,919,923,952,956,960,987,998,1116,1120,1245,1249,1253,1267,1318,1322,1326,1840,2104,2170,2839,2843,2850,2856,2864,2868,2875,3016,3073,3382,3386,4065,4279,4303,4312,4316,4320,4324,4328,4383],"2011).":[339,4384],"subtype":[343],"interstitial":[345],"pneumonia":[346],"chronic":[349,507],"progressive,":[351],"replacing":[352],"normal":[354,742,1300,3032],"lace-like":[355],"architecture":[357],"patchy,":[359],"hyperproliferative":[360],"fibrous":[361],"tissue":[362],"(Lederer":[363],"Martinez,":[365,394],"2018).":[366,519,591,924],"Current":[367],"only":[370,1487,4013,4073,4093,4353],"modestly":[371],"effective":[372],"do":[374],"not":[375,384,1532,1707,1870,1906,4012],"reverse":[376],"underlying":[378],"fibrosis,":[379],"lung":[381,871,912,4375],"transplantation":[382],"always":[385],"an":[386,1014,1130,1271,1277,1998,2030,2042,2142,2723,2881,2971,3207,4092],"option":[387],"(King":[388],"2014;":[391,399],"Lederer":[392],"Richeldi":[396],"Valapour":[400],"2020).":[403],"While":[404],"much":[405],"early":[408],"work":[409,480,706,1315,3364],"pathogenesis":[412,4212],"focused":[413],"unraveling":[415],"contributions":[417],"fibroblasts,":[419],"studies":[421,640,662,810,1027,4260,4350],"families":[423],"predisposition":[426],"argue":[429,641,664,4008],"key":[439],"drivers":[440,1063],"disease":[442,4336],"(Alder":[443,538,584],"2015;":[446,458,466,541,549,630,683],"Armanios":[447,542],"2007;":[450,545],"Bullard":[451],"2005;":[454,890],"Cogan":[455,546],"Haschek":[459],"Witschi,":[461],"1979;":[462],"Kropski":[463,550,627],"Maitra":[467],"2010;":[470,1246,1319,4317],"Thomas":[471],"2002;":[474,886],"Wang":[475],"2009).":[478,961],"Recent":[479],"mice":[482,978,1082,4367],"shows":[483,1675,1702,2242,2413,2427,3223,3250],"dysfunctional":[485],"putative":[493],"alveoli":[497],"(Barkauskas":[498],"2013),":[501],"can":[502,673,827,1916,2960,4044,4127,4234,4253,4269,4351],"lead":[503,2191],"progressive":[506,1523],"fibrotic":[508,616,4359],"similar":[510,1443,2770,3996],"seen":[513,2708,3999],"patients":[515,947],"(Nureki":[516],"Furthermore,":[520,1067,2905,3435,4361],"many":[521,2807,3001,3254,4160],"same":[524,2564,2597],"genes":[525,1705,1899,2679,2697,2927,2954,2974,3019,3076,3189,3282,3331,3415],"when":[527,966,1369,2680],"mutated":[528,1138],"cause":[529,647],"have":[531,612,940,2301,2341,2369,3599,3645,3689],"also":[532,663,784,1056,2879,2984,3458,4023,4051,4182,4254,4412],"linked":[534,569],"familial":[536],"2017).":[553,634,660,758,1268,3017,3074],"In":[554,759,1483],"sporadic":[555,605],"PF,":[556,601,854],"both":[557,3093,4271,4337],"age":[558,571],"factors,":[564,795],"risks":[567],"because":[570],"associated":[573,1210,2809,3231,3303,3313,3373,4099,4140,4162],"shortening":[576,1155,1344,1525,3068],"lung,":[579],"2008;":[587,957,2840,4325],"Everaerts":[588],"Consistent":[592,2135],"role":[595],"dysfunction":[598,667,698,787,847,1109,2147,2377,2532,2859,3371,3695,3823,4126],"driving":[600],"express":[607],"senescence":[610,669,1949,2498,3918],"shorter":[613,2088,4040],"regions":[617,622],"than":[618,2089,2116,4041],"those":[619,2643,3998,4042],"non-fibrotic":[621],"(Disayabutr":[623],"2016;":[626,679,754,878,3383],"Snetselaar":[631],"Additionally,":[635],"two":[636,3046,4239],"Mendelian":[638,655],"randomization":[639],"(Duckworth":[650],"2020;":[653,4380],"Telomeres":[654],"Randomization":[656],"Collaboration":[657],"Murine":[661],"drive":[674,853,935],"(Naikawadi":[676],"Povedano":[680],"Yao":[684],"2019).":[687,1121,4066],"Although":[688],"causality":[689],"thus":[691,3407],"evident,":[692],"exactly":[693],"leads":[699],"poorly":[703],"understood.":[704],"Previous":[705,1314],"our":[708,3362],"lab":[709],"using":[710,1403,1485,2064,2100,3746],"mouse":[711,1070,1086],"(iPS)":[717],"organoid":[719,1740,3768],"models":[720,3377,4392],"intestinal":[723,747,3376],"uncovered":[725],"positive":[727,2347,2508,2520,2535,3651,3699,3791,3807,3826],"feedback":[728],"loop":[729],"capping":[733,4059],"one":[739,927,2399,3728],"another":[740,3460],"under":[741,2446],"conditions":[743],"maintain":[745,3031],"niche":[750],"(Woo":[751,3380],"Yang":[755,3014,3071,3384],"setting":[761],"dysfunction,":[764],"virtuous":[766],"cycle":[767,2005,2165,2260],"becomes":[768],"vicious:":[769],"resulting":[771,1341],"suppression":[772],"interferes":[776],"function":[780,1113,2872,4222],"directly,":[781],"it":[783,1058,4022,4049],"amplifies":[785],"diminishing":[789],"Wnt-dependent":[790],"expression":[791,1166,1894,1975,2001,2233,2252,2663,2706],"catalytic":[798],"subunit":[799],"telomerase,":[801],"TERT,":[802],"several":[804],"telomere-protective":[807],"shelterins.":[808],"demonstrated":[811,2998],"pathway":[814,2766,2979,3006,3292],"(including":[816],"inhibitors,":[818,1177],"mimic":[820],"inhibition":[822,3368,3482,4011,4192],"Wnts)":[826],"defects,":[830,1960,4026],"raising":[831],"possibility":[833,1191],"agonism":[836,859],"therapeutic":[840],"benefit":[841,863,1198,4300],"DC.":[843],"Given":[844,3361,4331],"appears":[851,2433],"we":[855,1143,1232,1961,2657,2996,3388],"wondered":[856],"if":[857,3436],"might":[860,1110],"PF.":[865],"important":[869],"(Frank":[875],"Goss":[879],"2009;":[882],"Li":[883,887],"Maretto":[891],"2003;":[894,953,2171],"Okubo":[895],"Hogan,":[897],"2004;":[898],"Ostrin":[899],"Shu":[903],"2005)":[906],"regeneration":[908],"injury":[916],"(Nabhan":[917],"Zacharias":[921],"On":[925,962],"hand,":[928,965],"there":[929,1874,2266,4362],"evidence":[931],"Wnts":[933,1038],"may":[934,1195,3060,3121,3128],"PF;":[936],"example,":[938],"they":[939],"found":[942,2673,2798,2926,4133],"upregulated":[945],"(Chilosi":[950],"K\u00f6nigshoff":[954,958,4322],"other":[964,1540,2118,2755],"transcriptional":[970,3042],"effector":[971,3043],"\u03b2-catenin":[972,3409,4242,4247,4294],"deleted":[974],"sensitized":[980],"bleomcyin":[982],"(Tanjore":[985,4301],"2013).":[988],"complex":[992,4232],"context":[994,4225,4344],"(Wiese":[996],"2018),":[999,2851,2869],"exact":[1002],"spatial,":[1003,4397],"temporal,":[1004,4398],"cell-type":[1006],"specificity":[1007],"area":[1015,1639,1755,3858,3888],"intense":[1017],"investigation.":[1018],"It":[1019],"difficult":[1021],"extrapolate":[1023],"observational":[1025],"pathologic":[1026],"fully":[1029],"developed":[1030,1406,1451],"functional":[1035],"impact":[1036,1111],"at":[1039,1507,1699,1820,1852,1865,1950,1970,2131,2225,2238,2263,2496,2620,2686,2698,2709,2940,2948,3177,3191,3199,3271,3297,3320,3346],"earlier":[1040],"stages":[1041],"disease,":[1044],"large":[1047,2692],"number":[1048,1664,1744,1796,2144,2693,3185,3772],"interacting":[1050],"types":[1052],"lungs":[1055],"makes":[1057],"challenging":[1059],"identify":[1061],"primary":[1062],"such":[1065,1178,4266],"studies.":[1066],"differences":[1068,4365],"between":[1069,2245,4366],"biology":[1074,4376],"together":[1075],"generally":[1078],"lower":[1079,1457,3090,3601,3647,3691],"susceptibility":[1080],"make":[1085,4288],"modeling":[1087],"difficult.":[1088],"therefore":[1090,4287],"generated":[1091,1864],"directed":[1096],"differentiation":[1097,1415,1768,1817,2493,2551,2584,2625],"iPSCs":[1100,1427,1486],"(iPSC-derived":[1101],"[iAT2]":[1103],"cells)":[1104],"explore":[1106],"their":[1112,1182,2932,3288],"(Jacob":[1114,1265],"2017;":[1117],"Jacob":[1118,1408],"By":[1122],"comparing":[1123,2681,3201,3273,3322],"iAT2s":[1124,1437,1497,1814,2340,2368],"isogenic":[1127,1272,4108],"except":[1128],"introduced":[1131,1278,1903],"mutation":[1132,1220,1238,1309,1336,1435,1495,1548,1594,1714,1904,1945,2224],"gene":[1135,1165,1974,2662,2705],"most":[1136,2013,2117,4027],"often":[1137,4352],"DC,":[1140,2066],"X-linked":[1141],"DKC1,":[1142],"show":[1144,2227,3167,3182,4262],"become":[1149],"senescent":[1150,4155],"concert":[1152],"uncapping.":[1157],"short,":[1161],"exhibit":[1164,1337],"changes":[1167,2011,2651,2664,2707,2756,2771],"consistent":[1168,4090,4188],"decreased":[1170,1338],"dysfunction.":[1186,4228],"raise":[1189],"rescuing":[1200],"patients.":[1215],"Engineering":[1217],"DKC1":[1219,1240,1307,1334,1364,1529,1546,1592,1612,1676,1712,1855,2249,2296,2337,2365,2409,2421,3202,3274,3323,3342,3544,3594,3640,3684],"into":[1221,1255,1400,4238],"iPS":[1222,1262,1293,1330,1366,1397,1625,1914,1937,4110],"To":[1224,1954,2648],"engineered":[1233],"well-characterized,":[1235],"causal":[1236],"(DKC1":[1241],"A386T)":[1242],"(Agarwal":[1243,1316],"Batista":[1247,1320],"2011;":[1250,1323,4321],"Woo":[1251,1324],"2016)":[1254,1327],"BU3":[1257,1362],"NKX2.1::GFP":[1258],"SFTPC::TdTomato":[1259],"(NGST)":[1260],"established":[1270,1328],"pair":[1273],"lines:":[1276],"corresponding":[1283],"unedited":[1284,2831],"wild-type":[1285],"(WT)":[1286,3542],"(Figure":[1288,1310,1375,1481,1509,1884,1910,2018,2039,2050,2057,2092,2133,2670,2700,2887,2903,2922,2942,2981,3012,3069,3097,3416,3433,3453,3473,3956,3978,4003,4086],"1\u2014figure":[1289,1311,1376,1418,1510],"supplement":[1290,1312,1377,1419,1511,2059,2094,2571,2604,3099,3135,3455,3475,3980,4088],"1A-C).":[1291,2060],"Both":[1292],"lines":[1294,1399,4112],"maintained":[1295,1893],"markers":[1296,1991,2254,3919],"pluripotency":[1298],"karyotypes":[1301],"after":[1302,1430,1490,1526,1941,2220,3568,3793,3809,3828],"introduction":[1304,1432,1492,1942,2221],"A386T":[1308,1335,1365,1547,1593,1613,1677,1713,2250,2297,2338,2366,2410,2422,3203,3275,3324,3343,3545,3595,3641,3685],"1D).":[1313,1885],"passage.":[1346,2417],"confirmed":[1348,3078],"was":[1352,1477,1849,1869,2556,2589,2916,3860,3890,3986,4079],"reduced,":[1353],"successive":[1358,1580,1930,1978],"passages,":[1359],"NGST":[1363],"compared":[1370,1963],"WT":[1373,1965,2068,2091,2682,4001],"control":[1374],"2A-C).":[1378],"fail":[1384,1575],"alveolospheres":[1387,1578,1617,1679,1892,1923,1969,1986,2017,2028,2140,2412,3360,3437,3587,3633,3677,3926],"grow":[1389],"size":[1391,1506],"next":[1393],"differentiated":[1394],"paired":[1396],"protocol":[1405,1584,3524,3738,3843],"al.":[1410],"(see":[1411,2757,2789,2812],"Figure":[1412,1417,1551,1725,1732,1734,1761,1806,2172,2195,2465,2472,2474,2501,2513,2525,2540,2559,2569,2573,2592,2602,2606,3131,3490,3617,3661,3709,3753,3760,3762,3784,3800,3816,3862,3876,3892,3903,3911],"1A":[1413],"strategy,":[1416],"3A,":[1420],"B":[1421],"representative":[1423],"sorting":[1424],"strategies).":[1425],"Using":[1426],"35":[1428,1939,2218],"passages":[1429,1489,1701,1940,1979,2219],"yielded":[1436,1496],"initially":[1439],"grew":[1440],"fashion":[1444,3432],"WT,":[1446],"over":[1449,1801,1804,1815,1977,2491],"time":[1450,1867,3157,4341],"defect":[1454,1877,3397,3424,3450,4085,4143,4151],"alveolosphere":[1458,1503,1633,1638,3395,3422,3560,3565,4145,4199],"formation":[1459,1641,1750,3396,3566,3778,4146],"efficiency":[1460,1642,1751,3567,3779],"smaller":[1464],"alveolospheres.":[1465,3489,3521],"phenotype":[1467],"became":[1468],"50":[1471],"days":[1472,4075],"culture":[1474],"(D50)":[1475],"dramatic":[1478,1500],"D70":[1480,1508,1644,1853,2025,2132,2226,2264,2699,2710,2941,2949,3055,3181,3200,3272,3298,3321,3347,3443,3586,3632,3676,4078],"1A\u2013C).":[1482],"contrast,":[1484],"five":[1488],"less":[1499],"4A-C).":[1512],"indicate":[1515,2176,3103],"observed":[1519],"were":[1520,2012,3052,3077,3170,3438],"introducing":[1527],"mutation,":[1530],"immediate":[1534],"effects":[1535,1589],"deficiency":[1538],"(or":[1539],"deficiencies)":[1542],"per":[1549],"se.":[1550],"4":[1554,3491],"supplements":[1555,2199,3494],"see":[1556,2200,2823,3136,3495],"all":[1557,1632,2201,3137,3496,3559],"Download":[1558,1824,2202,2631,3138,3497,3914],"asset":[1559,1561,2203,2205,3139,3141,3498,3500],"Open":[1560,2204,3140,3499],"(iPSC)-derived":[1568],"(iAT2s)":[1574,1780,2217],"passaging.":[1581,1931],"(A)":[1582,2231,3163,3522],"Differentiation":[1583,3523],"used":[1585,2567,2600,3525,4107],"probe":[1587],"D.E.,":[1598],"definitive":[1599],"endoderm":[1600],"specification;":[1601],"Ant.,":[1602],"anteriorization.":[1603],"(B)":[1604,2293,3174,3535],"Representative":[1605,2406,3536],"images":[1606,3537],"differentiating":[1608,3539],"wild":[1609,1720,2246,3540],"bearing":[1615,3547],"(scale":[1618,3554],"bars,":[1619,2319,2359,2390,3555,3627,3671,3719],"100":[1620],"\u00b5m":[1621],"indicated":[1623],"NKX2.1+":[1627],"progenitors;":[1628],"mm":[1630,3557],"images).":[1634,3561],"(C)":[1635,2334,3193,3562],"Quantifications":[1636,3563,3906],"(n":[1645,1684,1715,2284,2310,2350,2381,2451,3575,3619,3663,3711,3750],"=":[1646,1685,1716,1719,1723,2285,2311,2351,2382,2452,3576,3620,3664,3712,3751],"4;":[1647,1686],"**":[1648,1687,3237,3259,3350],"p<0.01,":[1649,1688,3238,3260],"****":[1650,2287,3241,3263,3580],"p<0.0001,":[1651,2288,3242,3264,3581],"Student\u2019s":[1653,1690,2316,2387,3582,3624,3668,3716],"t-test).":[1654,1691,3583],"(D)":[1655,2362,3220,3584],"Quantification":[1656,2419],"percentage":[1659,1860],"SFTPC+":[1660,1666,1682,1696,1765,1798,1862,1887,1982,2667],"produced":[1668,1800],"passage":[1670,1952,2186],"fewer":[1681,3944,3949,3953],"(E)":[1692,2405,3247,3630],"RNA-seq":[1693,1812,2480,2633,3142,3221,3248],"sorted":[1695,1981,2666],"different":[1700,1971],"grossly":[1708],"affected":[1709,3112],"4,":[1717,2286,2312,2352,2383,3577],"W":[1718],"type,":[1721],"M":[1722],"mutant).":[1724],"1\u2014source":[1726],"Source":[1729,2469,3757],"1C-E.":[1733],"1C-SourceData-FormationEfficiency&OrganoidArea.pzfx":[1735],"\u2013":[1736,1763,1808,2476,2503,2515,2527,2542,2575,2608,3764,3786,3802,3818,3836,3864,3878,3894,3905],"Raw":[1737,1809,2477,2504,2516,2528,2609,3765,3787,3803,3819,3837,3865,3879,3895],"counts":[1738,1741,1810,2478,2505,2517,2529,2610,3766,3769,3788,3804,3820],"normalized":[1742,3770],"input":[1747,3775],"calculate":[1749,3777],"measurements":[1756],"along":[1757,1766,1785,2510,2522,2537,2549,2582,2623,2626,2753,2969,3780,3797,3813,3832],"statistical":[1759,2628,3782],"tests.":[1760,3783],"1D-SourceData-SFTPC%&SFTPCCumYield.pzfx":[1762],"Percentage":[1764],"sort":[1782],"flow":[1783],"plots":[1784,3176],"cumulative":[1787],"yield":[1788,1844],"surfactant":[1790],"protein":[1791,1827,2049,2732],"C":[1792,1828],"calculated":[1793],"experiment":[1803],"time.":[1805],"1E-SourceData-AT2Markers.pzfx":[1807],"specifically":[1818,2494],"looking":[1819,2495,2619],"markers.":[1822],"https://cdn.elifesciences.org/articles/64430/elife-64430-fig1-data1-v2.zip":[1823],"elife-64430-fig1-data1-v2.zip":[1825],"Surfactant":[1826],"(SFTPC)":[1829],"specific":[1833,1898,2497,4402],"marker":[1834],"(Kalina":[1838],"1992),":[1841],"SFTPC::TdTomato+":[1846],"(SFTPC+)":[1847],"reduced":[1850],"significantly":[1851,2676],"cultures,":[1857],"while":[1858,2262,3124],"each":[1866,2398,3727],"point":[1868],"different,":[1871],"suggesting":[1872,1900],"proliferation":[1881,1990,2256],"or":[1882,4305],"survival":[1883],"Sorted":[1886],"multiple":[1896,2777,3315],"did":[1905],"lineage":[1908],"specification":[1909],"1E).":[1911],"Thus,":[1912],"generate":[1917,4114],"alveolospheres;":[1920],"however,":[1921],"lose":[1924],"capacity":[1926],"self-renew":[1928],"derived":[1935],"develop":[1946],"late":[1951],"(D70)":[1953],"better":[1955,4209],"understand":[1956,2650,4210],"passages.":[1972],"RNA-seq-based":[1973,2661],"analyses":[1976,2023,2720,3197],"showed":[1987,2029,2071,2141,2880,3943],"decreases":[1988],"(MKI67":[1992],"MCM2)":[1994],"increase":[1999,2031,2054,2280,3962,4095],"inhibitor":[2006],"CDKN1A":[2007,2282],"(p21),":[2008],"pronounced":[2014],"2A).":[2019,2095],"Immunofluorescence":[2020],"microscopy":[2021],"based":[2022],"DNA":[2033,2160,2727],"damage":[2034,2161,2728],"marked":[2035,2800],"53BP1":[2037,2308,2326,2507,3790,3947],"foci":[2038,2149,2309,2327,2379,2534,3697,3825],"2B),":[2040],"increased":[2043,2143,4102],"fraction":[2044,2304,2344,2372,3602,3648,3692],"expressing":[2047],"p21":[2048,2349,2519,3650,3806],"2C),":[2051],"no":[2053,2072,2243,3612,3656,3704,3968],"apoptosis":[2056],"2\u2014figure":[2058,2093],"Measuring":[2061],"length":[2063,2078,2622,3966,4098],"qPCR,":[2065],"significant":[2073,2269,2279,2893,2987],"change":[2074,3159],"average":[2076,2082,3964],"passage,":[2080],"although":[2081],"lengths":[2084,2099,2426],"mutants":[2086],"trended":[2087],"However,":[2096],"measuring":[2097],"TeSLA":[2101,2407,2547,2580,2612,3958],"(Lai":[2102],"2017),":[2105,2857,3387],"more":[2108,2658],"sensitive":[2109],"detection":[2112],"techniques,":[2119],"revealed":[2120,2722,2769,2950,2985,3959],"had":[2125],"preponderance":[2127,2429],"2E\u2013F).":[2134],"this,":[2137,4190],"(TIFs),":[2150],"hallmark":[2152],"(i.e.,":[2156],"signal":[2159],"responses":[2162],"checkpoint":[2166],"arrest":[2167],"Takai":[2168],"2D).":[2173],"them":[2192],"senesce.":[2194],"senescence.":[2230],"Gene":[2232,2717,3194],"profiling":[2234],"D28":[2239,2687],"D50":[2241],"difference":[2244],"decrease":[2270,2952,3970],"MCM2":[2272],"MKI67":[2274],"(p21)":[2283],"DEseq2":[2289,3243,3265],"pairwise":[2290,3244,3266],"contrast":[2291,3245,3267],"statistics).":[2292,3246,3268],"At":[2294,2335,2363],"D70,":[2295,2336,2364,2439],"higher":[2303,2343,2371],"*":[2313,2353,2384,3578,3622,3666,3714],"p<0.05,":[2314,2354,2385,3236,3258,3349,3579,3623,3667,3715],"t-test;":[2317,2357,2388,3625,3669,3717],"scale":[2318,2358,2389,3626,3670,3718],"10":[2320,2360,2391,3628,3672,3720],"\u00b5m;":[2321,2392,3721],"insets":[2322,2393,3722],"highlight":[2323,2394,2444,3723,4387],"noted":[2329,2400,3729],"white":[2332,2403,3732],"arrowheads).":[2333,3733],"student\u2019s":[2356],"\u00b5m).":[2361,3629,3673],"(TIFs)":[2380],"TIFs,":[2397,3726],"arrowhead).":[2404],"shorten":[2415],"(F)":[2418,3269,3674],"approach":[2438],"red":[2440],"colored":[2441],"points":[2443],"1.6":[2448],"kb":[2449],"threshold":[2450],"2,":[2453],"\u2018shortest":[2454],"20%\u2019":[2455],"20th":[2458],"percentile":[2459],"length,":[2462],"Kb).":[2464],"2\u2014source":[2466],"2.":[2473],"2A-SourceData-iAT2-SenescentGeneExpression.pzfx":[2475],"related":[2499,2644,2736,2748,2821],"genes.":[2500],"2B-SourceData-53BP1PositiveCounts.pzfx":[2502],"statistics.":[2512,2524,2539,3799,3815],"2C-SourceData-p21PositiveCounts.pzfx":[2514],"2D-SourceData-TIFPhenotype.pzfx":[2526],"2E,F-SourceData-2020-08-31-TeSLABlotMerged-Cropped.tiff":[2541],"Uncropped":[2543,2576],"raw":[2544,2577],"image":[2545,2578],"blot":[2548,2565,2581,2598,2618,3838,3866,3880,3896],"showing":[2552,2585,3365,3857,3887],"where":[2553,2586],"crop":[2555,2588],"placed":[2557,2590],"2E.":[2560,2593],"4\u2014figure":[2570,2603,3454,3474,3979,4087],"3B.":[2572,2605],"2E,F-SourceData-2020-08-31-TeSLABlotMerged.tiff":[2574],"2E,F-SourceData-TeSLADKC1A386TAlongDiff.pzfx":[2607],"quant":[2613],"software":[2614],"cropped":[2617],"analysis.":[2629],"https://cdn.elifesciences.org/articles/64430/elife-64430-fig2-data1-v2.zip":[2630],"elife-64430-fig2-data1-v2.zip":[2632],"reveals":[2634,3155,3278,3294,3327],"pathways":[2635,2778,2808,4161],"differentially":[2636,2677,2695,3187],"expressed":[2637,2678,2696,3089,3188],"broadly":[2659],"evaluated":[2660],"3A).":[2671],"few":[2675],"D50,":[2689,3179],"3B),":[2701],"arguing":[2702],"likely":[2712,4028],"driven":[2713],"set":[2718,3195],"enrichment":[2719,3196],"(GSEA)":[2721,3198],"upregulation":[2724,2801,2882,2894,2972,3208,3224,3310,4030],"response,":[2729],"unfolded":[2731],"(UPR),":[2734],"mitochondrial":[2735,2858,4168],"functions":[2737],"(oxidative":[2738],"phosphorylation,":[2739],"respiratory":[2741],"electron":[2742],"chain),":[2743],"downregulation":[2745,2988,3252,3280,3300,3329],"hypoxia":[2747],"hedgehog":[2751],"Supplementary":[2758,2790,2813,2824],"file":[2759,2791,2814],"full":[2763,2795],"list).":[2764],"Ingenuity":[2765,3291],"(IPA)":[2768],"controlled":[2779,2955],"inflammatory":[2781],"cytokines":[2782],"like":[2783],"IL1\u03b2,":[2784],"IL6,":[2785],"IL17,":[2786],"others":[2788],"lists).":[2796],"3":[2815,3132,3493,3591,3637,3681],"curated":[2817],"list":[2818],"pathways,":[2822],"files":[2825],"analyses).":[2832],"included":[2834],"UPR":[2836],"(Lawson":[2837],"Mulugeta":[2841],"2005),":[2844],"thyroid":[2845,4172],"hormone":[2846,4173],"metabolism":[2847],"(Yu":[2848],"p53":[2852,4176],"(Shetty":[2854],"mitophagy":[2861],"(Chung":[2862],"2019;":[2865],"Yu":[2866],"caveolin":[2871],"(Wang":[2873],"2006).":[2876,4284],"non-canonical":[2884,2900,3118,3210,3229,4264],"3C),":[2888],"correlated":[2890],"WNT5A":[2896,3226],"WNT11,":[2898,3228],"known":[2899],"ligands":[2902],"3D).":[2904],"almost":[2906],"every":[2907],"FZD":[2908,3255],"gene,":[2909],"encoding":[2910,3020],"co-receptors":[2911],"downregulated":[2917,2935,3053,3081,3340],"3E).":[2923],"Also,":[2924],"GSEA":[2925,2983,3270,3319],"TCF7":[2929,2968,3284],"targets":[2930,2990,3037],"promoters":[2933],"3F).":[2943],"IPA":[2944],"master":[2946,3295],"regulators":[2947,3296],"lithium":[2957,3305],"chloride":[2958,3306],"(which":[2959],"potentiate":[2961],"inhibiting":[2965],"GSK3)":[2966],"usually":[2975],"stimulated":[2976],"inhibitors":[2980,3353],"3G).":[2982]},"counts_by_year":[],"updated_date":"2026-11-01T11:40:01.658920","created_date":"2022-06-16T00:00:00"}