{"id":"https://openalex.org/W2494915502","doi":"https://doi.org/10.15586/codon.pkd.2015.ch11","title":"The Role of G-protein Coupled Receptor Proteolytic Site (GPS) Cleavage in Polycystin-1 Biogenesis, Trafficking and Function","display_name":"The Role of G-protein Coupled Receptor Proteolytic Site (GPS) Cleavage in Polycystin-1 Biogenesis, Trafficking and Function","publication_year":2015,"publication_date":"2015-11-18","ids":{"openalex":"https://openalex.org/W2494915502","doi":"https://doi.org/10.15586/codon.pkd.2015.ch11","mag":"2494915502"},"language":"en","primary_location":{"id":"doi:10.15586/codon.pkd.2015.ch11","doi":"https://doi.org/10.15586/codon.pkd.2015.ch11","is_oa":true,"landing_page_url":"https://doi.org/10.15586/codon.pkd.2015.ch11","pdf_url":null,"source":{"id":"https://openalex.org/S4306486508","display_name":"Codon Publications eBooks","issn_l":null,"issn":null,"is_oa":false,"is_in_doaj":false,"is_core":false,"listed_in":[],"host_organization":"https://openalex.org/P4310318016","host_organization_name":"Codon Publications","host_organization_lineage":["https://openalex.org/P4310318016"],"host_organization_lineage_names":["Codon Publications"],"type":"ebook platform"},"license":"cc-by-nc","license_id":"https://openalex.org/licenses/cc-by-nc","version":"publishedVersion","is_accepted":true,"is_published":true,"raw_source_name":"Polycystic Kidney Disease","raw_type":"book-chapter"},"volume":{"id":"https://openalex.org/W2475072425","display_name":"Polycystic Kidney Disease"},"type":"book-chapter","indexed_in":["crossref"],"open_access":{"is_oa":true,"oa_status":"hybrid","oa_url":"https://doi.org/10.15586/codon.pkd.2015.ch11","any_repository_has_fulltext":false},"authorships":[{"author_position":"first","author":{"id":"https://openalex.org/A5100655901","display_name":"Feng Qian","orcid":"https://orcid.org/0000-0001-9595-9772","observed_orcids":["https://orcid.org/0000-0001-9595-9772"]},"institutions":[{"id":"https://openalex.org/I126744593","display_name":"University of Maryland, Baltimore","ror":"https://ror.org/04rq5mt64","country_code":"US","type":"education","lineage":["https://openalex.org/I126744593"]}],"countries":["US"],"is_corresponding":true,"raw_author_name":"Feng Qian","raw_affiliation_strings":["Department of Medicine, Division of Nephrology, University of Maryland School of Medicine, Baltimore, MD 21201, USA"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"Department of Medicine, Division of Nephrology, University of Maryland School of Medicine, Baltimore, MD 21201, USA","institution_ids":["https://openalex.org/I126744593"]}]}],"institutions":[],"countries_distinct_count":1,"institutions_distinct_count":1,"corresponding_author_ids":["https://openalex.org/A5100655901"],"corresponding_institution_ids":["https://openalex.org/I126744593"],"apc_list":null,"apc_paid":null,"fwci":0.9649,"has_fulltext":true,"cited_by_count":7,"citation_normalized_percentile":{"value":0.82447235,"is_in_top_1_percent":false,"is_in_top_10_percent":false},"cited_by_percentile_year":{"min":88,"max":95},"biblio":{"volume":null,"issue":null,"first_page":"259","last_page":"281"},"is_retracted":false,"is_paratext":false,"is_xpac":false,"primary_topic":{"id":"https://openalex.org/T11412","display_name":"Genetic and Kidney Cyst Diseases","score":1.0,"subfield":{"id":"https://openalex.org/subfields/1311","display_name":"Genetics"},"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"}},"topics":[{"id":"https://openalex.org/T11412","display_name":"Genetic and Kidney Cyst Diseases","score":1.0,"subfield":{"id":"https://openalex.org/subfields/1311","display_name":"Genetics"},"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/T10665","display_name":"Cystic Fibrosis Research Advances","score":0.0,"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/T10617","display_name":"Cellular transport and secretion","score":0.0,"subfield":{"id":"https://openalex.org/subfields/1307","display_name":"Cell 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"}}],"keywords":[{"id":"https://openalex.org/keywords/polycystin-1","display_name":"polycystin-1","score":0.9940000176429749},{"id":"https://openalex.org/keywords/pkd1","display_name":"PKD1","score":0.9110000133514404},{"id":"https://openalex.org/keywords/autosomal-dominant-polycystic-kidney-disease","display_name":"autosomal dominant polycystic kidney disease","score":0.9869999885559082},{"id":"https://openalex.org/keywords/polycystin-2","display_name":"polycystin-2","score":0.8809999823570251},{"id":"https://openalex.org/keywords/gga1","display_name":"GGA1","score":0.9039999842643738},{"id":"https://openalex.org/keywords/arl3","display_name":"ARL3","score":0.9539999961853027},{"id":"https://openalex.org/keywords/ciliary-trafficking","display_name":"ciliary trafficking","score":0.8960000276565552},{"id":"https://openalex.org/keywords/protein-biogenesis","display_name":"protein biogenesis","score":0.6880000233650208},{"id":"https://openalex.org/keywords/protein-trafficking","display_name":"protein trafficking","score":0.6010000109672546},{"id":"https://openalex.org/keywords/trans-golgi","display_name":"trans-Golgi","score":0.734000027179718},{"id":"https://openalex.org/keywords/heterodimer","display_name":"heterodimer","score":0.574999988079071},{"id":"https://openalex.org/keywords/cyst-formation","display_name":"cyst formation","score":0.7960000038146973},{"id":"https://openalex.org/keywords/distal-nephron","display_name":"distal nephron","score":0.781000018119812}],"concepts":[{"id":"https://openalex.org/C95444343","wikidata":"https://www.wikidata.org/wiki/Q7141","display_name":"Cell biology","level":1,"score":0.6782848834991455},{"id":"https://openalex.org/C1009742","wikidata":"https://www.wikidata.org/wiki/Q5333998","display_name":"Ectodomain","level":3,"score":0.6587320566177368},{"id":"https://openalex.org/C175156509","wikidata":"https://www.wikidata.org/wiki/Q1990218","display_name":"Cleavage (geology)","level":3,"score":0.6403672695159912},{"id":"https://openalex.org/C100094513","wikidata":"https://www.wikidata.org/wiki/Q180436","display_name":"Cilium","level":2,"score":0.6312593817710876},{"id":"https://openalex.org/C131934819","wikidata":"https://www.wikidata.org/wiki/Q135390","display_name":"Biogenesis","level":3,"score":0.5561778545379639},{"id":"https://openalex.org/C86803240","wikidata":"https://www.wikidata.org/wiki/Q420","display_name":"Biology","level":0,"score":0.505579948425293},{"id":"https://openalex.org/C119062480","wikidata":"https://www.wikidata.org/wiki/Q83181","display_name":"Golgi apparatus","level":3,"score":0.4739677608013153},{"id":"https://openalex.org/C57581600","wikidata":"https://www.wikidata.org/wiki/Q2449730","display_name":"Transport protein","level":2,"score":0.4339137673377991},{"id":"https://openalex.org/C185592680","wikidata":"https://www.wikidata.org/wiki/Q2329","display_name":"Chemistry","level":0,"score":0.3541911244392395},{"id":"https://openalex.org/C170493617","wikidata":"https://www.wikidata.org/wiki/Q208467","display_name":"Receptor","level":2,"score":0.31285715103149414},{"id":"https://openalex.org/C54355233","wikidata":"https://www.wikidata.org/wiki/Q7162","display_name":"Genetics","level":1,"score":0.23038196563720703},{"id":"https://openalex.org/C158617107","wikidata":"https://www.wikidata.org/wiki/Q79927","display_name":"Endoplasmic reticulum","level":2,"score":0.18134120106697083},{"id":"https://openalex.org/C104317684","wikidata":"https://www.wikidata.org/wiki/Q7187","display_name":"Gene","level":2,"score":0.12702271342277527},{"id":"https://openalex.org/C43369102","wikidata":"https://www.wikidata.org/wiki/Q2307625","display_name":"Fracture (geology)","level":2,"score":0.0},{"id":"https://openalex.org/C151730666","wikidata":"https://www.wikidata.org/wiki/Q7205","display_name":"Paleontology","level":1,"score":0.0}],"mesh":[],"locations_count":1,"locations":[{"id":"doi:10.15586/codon.pkd.2015.ch11","doi":"https://doi.org/10.15586/codon.pkd.2015.ch11","is_oa":true,"landing_page_url":"https://doi.org/10.15586/codon.pkd.2015.ch11","pdf_url":null,"source":{"id":"https://openalex.org/S4306486508","display_name":"Codon Publications eBooks","issn_l":null,"issn":null,"is_oa":false,"is_in_doaj":false,"is_core":false,"listed_in":[],"host_organization":"https://openalex.org/P4310318016","host_organization_name":"Codon Publications","host_organization_lineage":["https://openalex.org/P4310318016"],"host_organization_lineage_names":["Codon Publications"],"type":"ebook platform"},"license":"cc-by-nc","license_id":"https://openalex.org/licenses/cc-by-nc","version":"publishedVersion","is_accepted":true,"is_published":true,"raw_source_name":"Polycystic Kidney Disease","raw_type":"book-chapter"}],"best_oa_location":{"id":"doi:10.15586/codon.pkd.2015.ch11","doi":"https://doi.org/10.15586/codon.pkd.2015.ch11","is_oa":true,"landing_page_url":"https://doi.org/10.15586/codon.pkd.2015.ch11","pdf_url":null,"source":{"id":"https://openalex.org/S4306486508","display_name":"Codon Publications eBooks","issn_l":null,"issn":null,"is_oa":false,"is_in_doaj":false,"is_core":false,"listed_in":[],"host_organization":"https://openalex.org/P4310318016","host_organization_name":"Codon Publications","host_organization_lineage":["https://openalex.org/P4310318016"],"host_organization_lineage_names":["Codon Publications"],"type":"ebook platform"},"license":"cc-by-nc","license_id":"https://openalex.org/licenses/cc-by-nc","version":"publishedVersion","is_accepted":true,"is_published":true,"raw_source_name":"Polycystic Kidney Disease","raw_type":"book-chapter"},"sustainable_development_goals":[{"score":0.6240000128746033,"id":"https://metadata.un.org/sdg/3","display_name":"Good health and well-being"}],"study_designs":[],"awards":[{"id":"https://openalex.org/G3042977055","display_name":null,"funder_award_id":"DK090868","funder_id":"https://openalex.org/F4320337357","funder_display_name":"National Institute of Diabetes and Digestive and Kidney Diseases"},{"id":"https://openalex.org/G4008918121","display_name":null,"funder_award_id":"R01 DK062199","funder_id":"https://openalex.org/F4320306478","funder_display_name":"PKD Foundation"},{"id":"https://openalex.org/G4054591189","display_name":null,"funder_award_id":"P30 DK090868","funder_id":"https://openalex.org/F4320306478","funder_display_name":"PKD Foundation"},{"id":"https://openalex.org/G4248165597","display_name":null,"funder_award_id":"195G14a","funder_id":"https://openalex.org/F4320306478","funder_display_name":"PKD Foundation"},{"id":"https://openalex.org/G5332972337","display_name":"Baltimore Polycystic Kidney Disease Research and Clinical Core Center","funder_award_id":"P30DK090868","funder_id":"https://openalex.org/F4320337357","funder_display_name":"National Institute of Diabetes and Digestive and Kidney Diseases"}],"funders":[{"id":"https://openalex.org/F4320306478","display_name":"PKD Foundation","ror":"https://ror.org/05g0xkn45"},{"id":"https://openalex.org/F4320337357","display_name":"National Institute of Diabetes and Digestive and Kidney Diseases","ror":"https://ror.org/00adh9b73"}],"has_content":{"grobid_xml":false,"pdf":false},"content_urls":null,"referenced_works_count":78,"referenced_works":["https://openalex.org/W162961686","https://openalex.org/W1479807982","https://openalex.org/W1540558042","https://openalex.org/W1813497765","https://openalex.org/W1840138540","https://openalex.org/W1959400241","https://openalex.org/W1963767630","https://openalex.org/W1963934376","https://openalex.org/W1966685035","https://openalex.org/W1978100164","https://openalex.org/W1981137206","https://openalex.org/W1987219070","https://openalex.org/W1990249568","https://openalex.org/W1993655108","https://openalex.org/W1998357223","https://openalex.org/W2000538799","https://openalex.org/W2005026678","https://openalex.org/W2012867369","https://openalex.org/W2013039819","https://openalex.org/W2014604277","https://openalex.org/W2022169605","https://openalex.org/W2022215748","https://openalex.org/W2023172957","https://openalex.org/W2042641050","https://openalex.org/W2046518908","https://openalex.org/W2047052174","https://openalex.org/W2047578399","https://openalex.org/W2051209896","https://openalex.org/W2051339110","https://openalex.org/W2055211442","https://openalex.org/W2059957280","https://openalex.org/W2068642486","https://openalex.org/W2068801471","https://openalex.org/W2071104503","https://openalex.org/W2071302452","https://openalex.org/W2072199952","https://openalex.org/W2075591864","https://openalex.org/W2077431404","https://openalex.org/W2078277615","https://openalex.org/W2081659168","https://openalex.org/W2085346322","https://openalex.org/W2086655428","https://openalex.org/W2088811685","https://openalex.org/W2090905561","https://openalex.org/W2095381111","https://openalex.org/W2095708619","https://openalex.org/W2095869636","https://openalex.org/W2104243614","https://openalex.org/W2106182995","https://openalex.org/W2107175020","https://openalex.org/W2111689719","https://openalex.org/W2112591804","https://openalex.org/W2113588019","https://openalex.org/W2115850825","https://openalex.org/W2118200380","https://openalex.org/W2123474135","https://openalex.org/W2124708335","https://openalex.org/W2128870952","https://openalex.org/W2128969723","https://openalex.org/W2131999768","https://openalex.org/W2134844061","https://openalex.org/W2135100794","https://openalex.org/W2135389531","https://openalex.org/W2136875701","https://openalex.org/W2137621971","https://openalex.org/W2138967544","https://openalex.org/W2140871692","https://openalex.org/W2141331673","https://openalex.org/W2144246125","https://openalex.org/W2144273979","https://openalex.org/W2154643490","https://openalex.org/W2155338653","https://openalex.org/W2156427892","https://openalex.org/W2159434269","https://openalex.org/W2164020057","https://openalex.org/W2165899507","https://openalex.org/W2170339840","https://openalex.org/W2341400292"],"related_works":["https://openalex.org/W2283563018","https://openalex.org/W7097157263","https://openalex.org/W2068642486","https://openalex.org/W2982360022","https://openalex.org/W2509766347","https://openalex.org/W4308202827","https://openalex.org/W2078277615","https://openalex.org/W2154643490","https://openalex.org/W2135389531","https://openalex.org/W3015354305"],"abstract_inverted_index":{"Polycystin-1":[0],"(PC1)":[1],"is":[2,29,41,83,174,202],"encoded":[3],"by":[4,44],"PKD1,":[5],"the":[6,26,48,58,61,72,86,89,94,98,121,131,155,194,209,216,223,232,236,241,247],"principal":[7],"gene":[8],"mutated":[9],"in":[10,25,71,143,149,154,183,193,267],"autosomal":[11],"dominant":[12],"polycystic":[13],"kidney":[14,27],"disease":[15],"(ADPKD).":[16],"The":[17],"protein":[18],"regulates":[19],"terminal":[20],"differentiation":[21],"of":[22,39,60,74,81,88,104,130,179,206,234,249,264,269],"tubular":[23],"structures":[24],"and":[28,101,114,128,147,159,196,222,256,266],"required":[30,175,203],"to":[31,108,124,167,208],"maintain":[32],"their":[33],"structural":[34],"integrity.":[35],"A":[36],"fundamental":[37],"property":[38],"PC1":[40,82,105,144,171,188,207,253],"post-translational":[42],"modification":[43],"cis-autoproteolytic":[45],"cleavage":[46,65,80,138,152,251],"at":[47,57,120,215],"G-protein":[49],"coupled":[50],"receptor":[51],"proteolytic":[52],"site":[53],"(GPS)":[54],"motif":[55],"located":[56],"base":[59],"extracellular":[62],"ectodomain.":[63],"Defective":[64],"likely":[66],"plays":[67,139],"a":[68,140,169,184],"significant":[69],"role":[70,142],"pathogenesis":[73],"ADPKD.":[75,270],"In":[76,231],"mouse":[77],"models,":[78],"GPS":[79,137,151,250],"essential":[84],"for":[85,176,204,228,252,261],"integrity":[87],"distal":[90],"nephron":[91],"segments":[92],"during":[93],"postnatal":[95],"period.":[96],"While":[97],"exact":[99],"cellular":[100],"biochemical":[102],"functions":[103],"have":[106],"yet":[107],"be":[109,117],"fully":[110],"elucidated,":[111],"its":[112],"trafficking":[113,146,178,255],"function":[115,129,148,257],"must":[116],"precisely":[118],"regulated":[119],"subcellular":[122],"level":[123],"ensure":[125],"proper":[126],"structure":[127],"kidney.":[132],"Recent":[133],"evidence":[134],"shows":[135],"that":[136,258],"central":[141],"biogenesis,":[145],"vivo.":[150],"results":[153],"N-terminal":[156],"fragment":[157,161],"(PC1NTF)":[158],"C-terminal":[160],"(PC1CTF),":[162],"which":[163,181],"remain":[164],"non-covalently":[165],"associated":[166],"form":[168],"heterodimeric":[170],"molecule.":[172],"Cleavage":[173],"ciliary":[177,229],"PC1,":[180],"occurs":[182],"two-step":[185],"mechanism.":[186],"First,":[187],"interacts":[189],"with":[190],"polycystin-2":[191],"(PC2)":[192],"ER":[195],"binds":[197],"Rabep1.":[198],"PC1/2":[199,237],"complex":[200,238],"formation":[201],"transition":[205],"trans-Golgi":[210],"compartment.":[211],"Second,":[212],"once":[213],"arriving":[214],"trans-Golgi,":[217],"PC1/2-bound":[218],"Rabep1":[219],"recruits":[220],"GGA1":[221],"small":[224],"GTPase":[225],"Arl3":[226],"sequentially":[227],"targeting.":[230],"absence":[233],"cleavage,":[235],"cannot":[239],"reach":[240],"trans-Golgi.":[242],"This":[243],"article":[244],"will":[245],"discuss":[246],"roles":[248],"structure,":[254],"are":[259],"relevant":[260],"normal":[262],"activity":[263],"polycystin-1":[265],"cystogenesis":[268]},"counts_by_year":[{"year":2025,"cited_by_count":1},{"year":2024,"cited_by_count":2},{"year":2022,"cited_by_count":1},{"year":2020,"cited_by_count":2},{"year":2018,"cited_by_count":1}],"updated_date":"2026-11-04T10:54:05.112534","created_date":"2016-08-23T00:00:00"}
