{"id":"https://openalex.org/W69086284","doi":"https://doi.org/10.1007/978-1-59745-384-4_6","title":"Mutations in Surfactant Protein C and Interstitial Lung Disease","display_name":"Mutations in Surfactant Protein C and Interstitial Lung Disease","publication_year":2010,"publication_date":"2010-01-01","ids":{"openalex":"https://openalex.org/W69086284","doi":"https://doi.org/10.1007/978-1-59745-384-4_6","mag":"69086284"},"language":"en","primary_location":{"id":"doi:10.1007/978-1-59745-384-4_6","doi":"https://doi.org/10.1007/978-1-59745-384-4_6","is_oa":false,"landing_page_url":"https://doi.org/10.1007/978-1-59745-384-4_6","pdf_url":null,"source":{"id":"https://openalex.org/S4306463399","display_name":"Humana Press eBooks","issn_l":null,"issn":null,"is_oa":false,"is_in_doaj":false,"is_core":false,"listed_in":[],"host_organization":"https://openalex.org/P4310315801","host_organization_name":"Humana Press","host_organization_lineage":["https://openalex.org/P4310315801","https://openalex.org/P4310319900"],"host_organization_lineage_names":["Humana Press","Springer Science+Business Media"],"type":"ebook platform"},"license":null,"license_id":null,"version":"publishedVersion","is_accepted":true,"is_published":true,"raw_source_name":"Molecular Basis of Pulmonary Disease","raw_type":"book-chapter"},"volume":{"id":"https://openalex.org/W2490314326","display_name":"Molecular Basis of Pulmonary Disease"},"type":"book-chapter","indexed_in":["crossref"],"open_access":{"is_oa":false,"oa_status":"closed","oa_url":null,"any_repository_has_fulltext":false},"authorships":[{"author_position":"first","author":{"id":"https://openalex.org/A5067641826","display_name":"Ralph J. Panos","orcid":"https://orcid.org/0000-0003-4978-7599","observed_orcids":["https://orcid.org/0000-0003-4978-7599"]},"institutions":[{"id":"https://openalex.org/I4210143084","display_name":"Cincinnati VA Medical Center","ror":"https://ror.org/045r80n66","country_code":"US","type":"healthcare","lineage":["https://openalex.org/I1322918889","https://openalex.org/I2799886695","https://openalex.org/I4210127612","https://openalex.org/I4210143084"]},{"id":"https://openalex.org/I63135867","display_name":"University of Cincinnati","ror":"https://ror.org/01e3m7079","country_code":"US","type":"education","lineage":["https://openalex.org/I63135867"]}],"countries":["US"],"is_corresponding":false,"raw_author_name":"Ralph J. Panos","raw_affiliation_strings":["Department of Internal Medicine, University of Cincinnati School of Medicine, Cincinnati VA Medical Center, Cincinnati, OH, USA"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"Department of Internal Medicine, University of Cincinnati School of Medicine, Cincinnati VA Medical Center, Cincinnati, OH, USA","institution_ids":["https://openalex.org/I4210143084","https://openalex.org/I63135867"]}]},{"author_position":"last","author":{"id":"https://openalex.org/A5050169529","display_name":"James P. Bridges","orcid":"https://orcid.org/0000-0002-4815-117X","observed_orcids":["https://orcid.org/0000-0002-4815-117X"]},"institutions":[{"id":"https://openalex.org/I1285204247","display_name":"Cincinnati Children's Hospital Medical Center","ror":"https://ror.org/01hcyya48","country_code":"US","type":"healthcare","lineage":["https://openalex.org/I1285204247"]}],"countries":["US"],"is_corresponding":false,"raw_author_name":"James P. Bridges","raw_affiliation_strings":["Department of Neonatology in Pulmonary Biology, Children\u2019s Hospital Medical Center, Cincinnati, OH, USA"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"Department of Neonatology in Pulmonary Biology, Children\u2019s Hospital Medical Center, Cincinnati, OH, USA","institution_ids":["https://openalex.org/I1285204247"]}]}],"institutions":[],"countries_distinct_count":1,"institutions_distinct_count":3,"corresponding_author_ids":[],"corresponding_institution_ids":[],"apc_list":null,"apc_paid":null,"fwci":1.9157,"has_fulltext":false,"cited_by_count":1,"citation_normalized_percentile":{"value":0.84345634,"is_in_top_1_percent":false,"is_in_top_10_percent":false},"cited_by_percentile_year":null,"biblio":{"volume":null,"issue":null,"first_page":"133","last_page":"166"},"is_retracted":false,"is_paratext":false,"is_xpac":false,"primary_topic":{"id":"https://openalex.org/T10870","display_name":"Interstitial Lung Diseases and Idiopathic Pulmonary Fibrosis","score":0.9994999766349792,"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"}},"topics":[{"id":"https://openalex.org/T10870","display_name":"Interstitial Lung Diseases and Idiopathic Pulmonary Fibrosis","score":0.9994999766349792,"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"}},{"id":"https://openalex.org/T10549","display_name":"Neonatal Respiratory Health Research","score":9.999999747378752e-05,"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"}},{"id":"https://openalex.org/T11026","display_name":"Occupational and environmental lung diseases","score":9.999999747378752e-05,"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/surfactant-protein-c","display_name":"surfactant protein C","score":0.9860000014305115},{"id":"https://openalex.org/keywords/idiopathic-pulmonary-fibrosis","display_name":"idiopathic pulmonary fibrosis","score":0.8629999756813049},{"id":"https://openalex.org/keywords/idiopathic-interstitial-lung-disease","display_name":"idiopathic interstitial lung disease","score":0.8849999904632568},{"id":"https://openalex.org/keywords/familial-pulmonary-fibrosis","display_name":"familial pulmonary fibrosis","score":0.9919999837875366},{"id":"https://openalex.org/keywords/alveolar-type-ii-cells","display_name":"alveolar type II cells","score":0.824999988079071},{"id":"https://openalex.org/keywords/lamellar-bodies","display_name":"lamellar bodies","score":0.8600000143051147},{"id":"https://openalex.org/keywords/proprotein-processing","display_name":"proprotein processing","score":0.6370000243186951},{"id":"https://openalex.org/keywords/intramolecular-chaperone","display_name":"intramolecular chaperone","score":0.753000020980835},{"id":"https://openalex.org/keywords/endoplasmic-reticulum-stress","display_name":"endoplasmic reticulum stress","score":0.828000009059906},{"id":"https://openalex.org/keywords/unfolded-protein-response","display_name":"unfolded protein response","score":0.9940000176429749},{"id":"https://openalex.org/keywords/proteasome-dysfunction","display_name":"proteasome dysfunction","score":0.9829999804496765},{"id":"https://openalex.org/keywords/epithelial-cell-death","display_name":"epithelial cell death","score":0.8320000171661377},{"id":"https://openalex.org/keywords/pulmonary-alveolar-proteinosis","display_name":"pulmonary alveolar proteinosis","score":0.8140000104904175},{"id":"https://openalex.org/keywords/nonspecific-interstitial-pneumonia","display_name":"nonspecific interstitial pneumonia","score":0.9070000052452087},{"id":"https://openalex.org/keywords/usual-interstitial-pneumonia","display_name":"usual interstitial pneumonia","score":0.9599999785423279},{"id":"https://openalex.org/keywords/autosomal-dominant-inheritance","display_name":"autosomal dominant inheritance","score":0.8849999904632568},{"id":"https://openalex.org/keywords/variable-penetrance","display_name":"variable penetrance","score":0.7170000076293945},{"id":"https://openalex.org/keywords/pathogenesis","display_name":"pathogenesis","score":0.527999997138977}],"concepts":[{"id":"https://openalex.org/C2778729956","wikidata":"https://www.wikidata.org/wiki/Q7646058","display_name":"Surfactant protein C","level":3,"score":0.9223474264144897},{"id":"https://openalex.org/C2778341716","wikidata":"https://www.wikidata.org/wiki/Q2290446","display_name":"Idiopathic pulmonary fibrosis","level":3,"score":0.7572951316833496},{"id":"https://openalex.org/C2777543607","wikidata":"https://www.wikidata.org/wiki/Q1153419","display_name":"Interstitial lung disease","level":3,"score":0.7199348211288452},{"id":"https://openalex.org/C2778972612","wikidata":"https://www.wikidata.org/wiki/Q448698","display_name":"Pulmonary alveolar proteinosis","level":3,"score":0.6647953987121582},{"id":"https://openalex.org/C145156650","wikidata":"https://www.wikidata.org/wiki/Q4253549","display_name":"Lamellar granule","level":3,"score":0.6439642906188965},{"id":"https://openalex.org/C200544954","wikidata":"https://www.wikidata.org/wiki/Q516389","display_name":"Penetrance","level":4,"score":0.6297712326049805},{"id":"https://openalex.org/C2781244666","wikidata":"https://www.wikidata.org/wiki/Q32446","display_name":"Pulmonary fibrosis","level":3,"score":0.5661248564720154},{"id":"https://openalex.org/C142724271","wikidata":"https://www.wikidata.org/wiki/Q7208","display_name":"Pathology","level":1,"score":0.5221136808395386},{"id":"https://openalex.org/C71924100","wikidata":"https://www.wikidata.org/wiki/Q11190","display_name":"Medicine","level":0,"score":0.5132091045379639},{"id":"https://openalex.org/C58226133","wikidata":"https://www.wikidata.org/wiki/Q1324199","display_name":"Pulmonary surfactant","level":2,"score":0.44576066732406616},{"id":"https://openalex.org/C2777714996","wikidata":"https://www.wikidata.org/wiki/Q7886","display_name":"Lung","level":2,"score":0.4425649642944336},{"id":"https://openalex.org/C2779524853","wikidata":"https://www.wikidata.org/wiki/Q1284347","display_name":"Pneumonitis","level":3,"score":0.4169999659061432},{"id":"https://openalex.org/C2780559512","wikidata":"https://www.wikidata.org/wiki/Q605709","display_name":"Fibrosis","level":2,"score":0.36813902854919434},{"id":"https://openalex.org/C203014093","wikidata":"https://www.wikidata.org/wiki/Q101929","display_name":"Immunology","level":1,"score":0.34723466634750366},{"id":"https://openalex.org/C86803240","wikidata":"https://www.wikidata.org/wiki/Q420","display_name":"Biology","level":0,"score":0.34281039237976074},{"id":"https://openalex.org/C126322002","wikidata":"https://www.wikidata.org/wiki/Q11180","display_name":"Internal medicine","level":1,"score":0.22024449706077576},{"id":"https://openalex.org/C54355233","wikidata":"https://www.wikidata.org/wiki/Q7162","display_name":"Genetics","level":1,"score":0.17905846238136292},{"id":"https://openalex.org/C104317684","wikidata":"https://www.wikidata.org/wiki/Q7187","display_name":"Gene","level":2,"score":0.1779012382030487},{"id":"https://openalex.org/C127716648","wikidata":"https://www.wikidata.org/wiki/Q104053","display_name":"Phenotype","level":3,"score":0.11610496044158936},{"id":"https://openalex.org/C55493867","wikidata":"https://www.wikidata.org/wiki/Q7094","display_name":"Biochemistry","level":1,"score":0.0669066309928894}],"mesh":[],"locations_count":1,"locations":[{"id":"doi:10.1007/978-1-59745-384-4_6","doi":"https://doi.org/10.1007/978-1-59745-384-4_6","is_oa":false,"landing_page_url":"https://doi.org/10.1007/978-1-59745-384-4_6","pdf_url":null,"source":{"id":"https://openalex.org/S4306463399","display_name":"Humana Press eBooks","issn_l":null,"issn":null,"is_oa":false,"is_in_doaj":false,"is_core":false,"listed_in":[],"host_organization":"https://openalex.org/P4310315801","host_organization_name":"Humana Press","host_organization_lineage":["https://openalex.org/P4310315801","https://openalex.org/P4310319900"],"host_organization_lineage_names":["Humana Press","Springer Science+Business Media"],"type":"ebook platform"},"license":null,"license_id":null,"version":"publishedVersion","is_accepted":true,"is_published":true,"raw_source_name":"Molecular Basis of Pulmonary Disease","raw_type":"book-chapter"}],"best_oa_location":null,"sustainable_development_goals":[{"score":0.9537000060081482,"id":"https://metadata.un.org/sdg/3","display_name":"Good health and well-being"}],"sustainable_development_goals_aurora":[{"score":0.5699999928474426,"id":"https://metadata.un.org/sdg/3","display_name":"Good health and well-being"}],"study_designs":[],"awards":[],"funders":[],"has_content":{"pdf":false,"grobid_xml":false},"content_urls":null,"referenced_works_count":217,"referenced_works":["https://openalex.org/W1224352","https://openalex.org/W29580419","https://openalex.org/W100839948","https://openalex.org/W121287568","https://openalex.org/W236234631","https://openalex.org/W1498451757","https://openalex.org/W1501290006","https://openalex.org/W1523677551","https://openalex.org/W1549251297","https://openalex.org/W1551952768","https://openalex.org/W1594764193","https://openalex.org/W1672219033","https://openalex.org/W1694680795","https://openalex.org/W1710057076","https://openalex.org/W1768877551","https://openalex.org/W1806301233","https://openalex.org/W1829364146","https://openalex.org/W1906404903","https://openalex.org/W1924766221","https://openalex.org/W1964706050","https://openalex.org/W1965635977","https://openalex.org/W1966959414","https://openalex.org/W1969758724","https://openalex.org/W1969901425","https://openalex.org/W1969981616","https://openalex.org/W1970171594","https://openalex.org/W1970255441","https://openalex.org/W1972374862","https://openalex.org/W1973023261","https://openalex.org/W1977481343","https://openalex.org/W1978134704","https://openalex.org/W1979994166","https://openalex.org/W1981312930","https://openalex.org/W1981551685","https://openalex.org/W1981625315","https://openalex.org/W1983357763","https://openalex.org/W1984672567","https://openalex.org/W1988103018","https://openalex.org/W1988297845","https://openalex.org/W1989728523","https://openalex.org/W1990584310","https://openalex.org/W1992938929","https://openalex.org/W1993559550","https://openalex.org/W1995301423","https://openalex.org/W1995517904","https://openalex.org/W1995740317","https://openalex.org/W1996730427","https://openalex.org/W1996819436","https://openalex.org/W1998579506","https://openalex.org/W1998621493","https://openalex.org/W1998975199","https://openalex.org/W1999644843","https://openalex.org/W2005537440","https://openalex.org/W2005543326","https://openalex.org/W2007012469","https://openalex.org/W2007109726","https://openalex.org/W2007697123","https://openalex.org/W2009713659","https://openalex.org/W2011225090","https://openalex.org/W2011870650","https://openalex.org/W2013114941","https://openalex.org/W2013465063","https://openalex.org/W2014811196","https://openalex.org/W2015607380","https://openalex.org/W2016204831","https://openalex.org/W2016354485","https://openalex.org/W2017139263","https://openalex.org/W2018429730","https://openalex.org/W2018809578","https://openalex.org/W2021069791","https://openalex.org/W2022252318","https://openalex.org/W2022898731","https://openalex.org/W2023879427","https://openalex.org/W2031074041","https://openalex.org/W2033324724","https://openalex.org/W2033846213","https://openalex.org/W2034686041","https://openalex.org/W2038447078","https://openalex.org/W2038482344","https://openalex.org/W2039075718","https://openalex.org/W2039778129","https://openalex.org/W2040355401","https://openalex.org/W2044478331","https://openalex.org/W2045076536","https://openalex.org/W2048456744","https://openalex.org/W2049557850","https://openalex.org/W2050171843","https://openalex.org/W2051772841","https://openalex.org/W2051879911","https://openalex.org/W2054159211","https://openalex.org/W2056497731","https://openalex.org/W2057458118","https://openalex.org/W2058448098","https://openalex.org/W2059618188","https://openalex.org/W2060126259","https://openalex.org/W2060763719","https://openalex.org/W2060928333","https://openalex.org/W2061932166","https://openalex.org/W2063042519","https://openalex.org/W2063902734","https://openalex.org/W2064609154","https://openalex.org/W2065254446","https://openalex.org/W2070537057","https://openalex.org/W2071128893","https://openalex.org/W2071229169","https://openalex.org/W2072063652","https://openalex.org/W2073188615","https://openalex.org/W2073727172","https://openalex.org/W2074190785","https://openalex.org/W2075216266","https://openalex.org/W2076880493","https://openalex.org/W2077039635","https://openalex.org/W2077462602","https://openalex.org/W2080534127","https://openalex.org/W2081347471","https://openalex.org/W2081906687","https://openalex.org/W2082144035","https://openalex.org/W2085295869","https://openalex.org/W2087585030","https://openalex.org/W2089157650","https://openalex.org/W2089198803","https://openalex.org/W2090819119","https://openalex.org/W2091114785","https://openalex.org/W2092126563","https://openalex.org/W2092564170","https://openalex.org/W2095054225","https://openalex.org/W2095947403","https://openalex.org/W2096033564","https://openalex.org/W2096879602","https://openalex.org/W2097419822","https://openalex.org/W2099049651","https://openalex.org/W2100059970","https://openalex.org/W2100880370","https://openalex.org/W2103327160","https://openalex.org/W2104558481","https://openalex.org/W2104927376","https://openalex.org/W2107051779","https://openalex.org/W2107367358","https://openalex.org/W2107665433","https://openalex.org/W2108226118","https://openalex.org/W2108683967","https://openalex.org/W2111369447","https://openalex.org/W2112286580","https://openalex.org/W2115176808","https://openalex.org/W2116000536","https://openalex.org/W2116488073","https://openalex.org/W2117015911","https://openalex.org/W2117257160","https://openalex.org/W2120997345","https://openalex.org/W2121453721","https://openalex.org/W2122738080","https://openalex.org/W2125322938","https://openalex.org/W2127942339","https://openalex.org/W2130098255","https://openalex.org/W2130104463","https://openalex.org/W2133284260","https://openalex.org/W2133937594","https://openalex.org/W2134215992","https://openalex.org/W2136062886","https://openalex.org/W2136114918","https://openalex.org/W2136616179","https://openalex.org/W2136761840","https://openalex.org/W2139373056","https://openalex.org/W2140881215","https://openalex.org/W2142382161","https://openalex.org/W2142941142","https://openalex.org/W2144017344","https://openalex.org/W2147342957","https://openalex.org/W2148738605","https://openalex.org/W2149476949","https://openalex.org/W2150824191","https://openalex.org/W2150912228","https://openalex.org/W2150943858","https://openalex.org/W2151320720","https://openalex.org/W2157869208","https://openalex.org/W2159852406","https://openalex.org/W2160001887","https://openalex.org/W2160856572","https://openalex.org/W2162809912","https://openalex.org/W2163779649","https://openalex.org/W2164390141","https://openalex.org/W2165771235","https://openalex.org/W2165871186","https://openalex.org/W2167472479","https://openalex.org/W2168228140","https://openalex.org/W2168236606","https://openalex.org/W2170296643","https://openalex.org/W2176158530","https://openalex.org/W2187434353","https://openalex.org/W2223090084","https://openalex.org/W2229805806","https://openalex.org/W2253617528","https://openalex.org/W2273415510","https://openalex.org/W2300202875","https://openalex.org/W2308212346","https://openalex.org/W2315096416","https://openalex.org/W2317552035","https://openalex.org/W2320667912","https://openalex.org/W2336127133","https://openalex.org/W2338428463","https://openalex.org/W2338532680","https://openalex.org/W2347145398","https://openalex.org/W2400830283","https://openalex.org/W2407213763","https://openalex.org/W2409002712","https://openalex.org/W2410416170","https://openalex.org/W2412481834","https://openalex.org/W2414784657","https://openalex.org/W2416349112","https://openalex.org/W2416432012","https://openalex.org/W2419604145","https://openalex.org/W2626588662","https://openalex.org/W2740355891","https://openalex.org/W2842267605","https://openalex.org/W3027658541","https://openalex.org/W4235251147","https://openalex.org/W6609078652"],"related_works":["https://openalex.org/W1966959414","https://openalex.org/W4316171344","https://openalex.org/W2885133358","https://openalex.org/W2107367358","https://openalex.org/W2149476949","https://openalex.org/W2136114918","https://openalex.org/W2150943858","https://openalex.org/W2015416553","https://openalex.org/W2585138611","https://openalex.org/W4410273098"],"abstract_inverted_index":{"Less":[0],"than":[1],"5%":[2],"of":[3,6,21,86,91,108,125,130,144,151,196,221,287,311],"all":[4],"cases":[5],"idiopathic":[7,33,313],"interstitial":[8,217,228,314],"lung":[9,241,315],"disease":[10],"(ILD)":[11],"are":[12,25],"due":[13],"to":[14,117,134,178,188,199,274],"familial":[15,22,53],"pulmonary":[16,23,34,222,300],"fibrosis.":[17,35,289],"The":[18,127,180,194],"clinical":[19,181],"manifestations":[20,182],"fibrosis":[24,242,301],"indistinguishable":[26],"from":[27,176,184],"the":[28,40,71,77,81,84,105,118,121,131,145,149,210,231,247,251,285,291,306,312],"presenting":[29],"symptoms":[30],"in":[31,37,50,73,174,235,297],"sporadic":[32,60],"Mutations":[36],"SFTPC":[38,163],",":[39],"gene":[41],"encoding":[42],"surfactant":[43],"protein":[44,66,147,244,253],"C":[45],"(SP-C),":[46],"have":[47,165],"been":[48,166],"identified":[49,167],"kindreds":[51],"with":[52,59,154,170,203,219],"ILD":[54,155],"as":[55,57,98,113,136],"well":[56],"individuals":[58,169],"IPF.":[61],"SP-C":[62,95,146,171,267,298],"is":[63,68,96,102,115,215,230],"a":[64,99],"surfactant-associated":[65],"that":[67,101,140,277],"essential":[69],"for":[70],"reduction":[72],"surface":[74],"tension":[75],"at":[76],"air\u2013liquid":[78],"interface":[79],"within":[80,104,157,246],"alveolus":[82],"and":[83,148,168,208,257,284,308],"prevention":[85],"end-expiratory":[87],"alveolar":[88,109,223,258],"collapse.":[89],"Because":[90],"its":[92],"hydrophobic":[93],"properties,":[94],"synthesized":[97],"proprotein":[100,132],"processed":[103],"secretory":[106],"pathway":[107],"type":[110,263],"II":[111,264],"cells":[112,265],"it":[114],"conducted":[116],"lamellar":[119],"body,":[120],"intracellular":[122],"storage":[123],"site":[124],"surfactant.":[126],"carboxy":[128],"terminus":[129],"appears":[133,198],"function":[135],"an":[137],"intramolecular":[138],"chaperone":[139],"guides":[141],"posttranslational":[142],"processing":[143],"majority":[150],"mutations":[152,164,172,238,296],"associated":[153],"occur":[156],"this":[158],"domain.":[159],"Over":[160],"50":[161],"distinct":[162],"range":[173],"age":[175],"infants":[177,207],"adults.":[179,236],"extend":[183],"fatal":[185],"respiratory":[186,192],"failure":[187],"no":[189],"clinically":[190],"apparent":[191],"symptoms.":[193],"pattern":[195,214,234],"inheritance":[197],"be":[200,271],"autosomal":[201],"dominant":[202],"variable":[204],"penetrance.":[205],"In":[206,225],"children,":[209],"most":[211,232],"common":[212],"histopathological":[213],"nonspecific":[216],"pneumonitis":[218,229],"features":[220],"proteinosis.":[224],"contrast,":[226],"usual":[227],"frequent":[233],"These":[237],"may":[239,270,278],"cause":[240,299],"through":[243],"misprocessing":[245],"endoplasmic":[248],"reticulum":[249],"activating":[250],"unfolded":[252],"response,":[254],"proteasome":[255],"dysfunction,":[256],"epithelial":[259,280],"cell":[260,281],"death.":[261],"Alveolar":[262],"expressing":[266],"mutant":[268],"proteins":[269],"more":[272],"susceptible":[273],"environmental":[275],"factors":[276],"trigger":[279],"injury,":[282],"death,":[283],"development":[286],"parenchymal":[288],"Understanding":[290],"pathogenetic":[292],"mechanisms":[293],"by":[294],"which":[295],"provides":[302],"unique":[303],"insights":[304],"into":[305],"cellular":[307],"molecular":[309],"pathogenesis":[310],"diseases.":[316]},"counts_by_year":[],"updated_date":"2026-10-30T12:39:25.321706","created_date":"2016-06-24T00:00:00"}