{"id":"https://openalex.org/W2158024088","doi":"https://doi.org/10.3390/pharmaceutics3040782","title":"Role of P-Glycoprotein Expression and Function in Cystinotic Renal Proximal Tubular Cells","display_name":"Role of P-Glycoprotein Expression and Function in Cystinotic Renal Proximal Tubular Cells","publication_year":2011,"publication_date":"2011-10-27","ids":{"openalex":"https://openalex.org/W2158024088","doi":"https://doi.org/10.3390/pharmaceutics3040782","mag":"2158024088","pmid":"https://pubmed.ncbi.nlm.nih.gov/24309308","pmcid":"https://www.ncbi.nlm.nih.gov/pmc/articles/3857058"},"language":"en","primary_location":{"id":"doi:10.3390/pharmaceutics3040782","doi":"https://doi.org/10.3390/pharmaceutics3040782","is_oa":true,"landing_page_url":"https://doi.org/10.3390/pharmaceutics3040782","pdf_url":"https://www.mdpi.com/1999-4923/3/4/782/pdf?version=1319718870","source":{"id":"https://openalex.org/S30283931","display_name":"Pharmaceutics","issn_l":"1999-4923","issn":["1999-4923"],"is_oa":true,"is_in_doaj":true,"is_core":true,"listed_in":["cwts-core","doaj","norway-1","poland-100","russia-white-list-1","vabb-shw"],"host_organization":"https://openalex.org/P4310310987","host_organization_name":"Multidisciplinary Digital Publishing Institute","host_organization_lineage":["https://openalex.org/P4310310987"],"host_organization_lineage_names":["Multidisciplinary Digital Publishing Institute"],"type":"journal"},"license":"cc-by","license_id":"https://openalex.org/licenses/cc-by","version":"publishedVersion","is_accepted":true,"is_published":true,"raw_source_name":"Pharmaceutics","raw_type":"journal-article"},"volume":null,"type":"article","indexed_in":["crossref","doaj","pubmed"],"open_access":{"is_oa":true,"oa_status":"gold","oa_url":"https://www.mdpi.com/1999-4923/3/4/782/pdf?version=1319718870","any_repository_has_fulltext":true},"authorships":[{"author_position":"first","author":{"id":"https://openalex.org/A5102857854","display_name":"Karen Peeters","orcid":"https://orcid.org/0000-0002-1166-2267","observed_orcids":["https://orcid.org/0000-0002-1166-2267"]},"institutions":[{"id":"https://openalex.org/I99464096","display_name":"KU Leuven","ror":"https://ror.org/05f950310","country_code":"BE","type":"education","lineage":["https://openalex.org/I99464096"]}],"countries":["BE"],"is_corresponding":false,"raw_author_name":"Karen Peeters","raw_affiliation_strings":["Pediatric Nephrology, Katholieke Universiteit Leuven, Herestraat 49 Box 817, Leuven, Belgium"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"Pediatric Nephrology, Katholieke Universiteit Leuven, Herestraat 49 Box 817, Leuven, Belgium","institution_ids":["https://openalex.org/I99464096"]}]},{"author_position":"middle","author":{"id":"https://openalex.org/A5036441925","display_name":"Martijn J. G. Wilmer","orcid":"https://orcid.org/0000-0003-4832-105X","observed_orcids":["https://orcid.org/0000-0003-4832-105X"]},"institutions":[{"id":"https://openalex.org/I145872427","display_name":"Radboud University Nijmegen","ror":"https://ror.org/016xsfp80","country_code":"NL","type":"education","lineage":["https://openalex.org/I145872427"]},{"id":"https://openalex.org/I2802934949","display_name":"Radboud University Medical Center","ror":"https://ror.org/05wg1m734","country_code":"NL","type":"funder","lineage":["https://openalex.org/I2802934949"]}],"countries":["NL"],"is_corresponding":false,"raw_author_name":"Martijn J. Wilmer","raw_affiliation_strings":["Department of Pharmacology and Toxicology (149), Radboud University Nijmegen Medical Center, Geert Grooteplein Noord 21, 6525 GA, Nijmegen, The Netherlands"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"Department of Pharmacology and Toxicology (149), Radboud University Nijmegen Medical Center, Geert Grooteplein Noord 21, 6525 GA, Nijmegen, The Netherlands","institution_ids":["https://openalex.org/I145872427","https://openalex.org/I2802934949"]}]},{"author_position":"middle","author":{"id":"https://openalex.org/A5011136623","display_name":"Joost P. H. Schoeber","orcid":null,"observed_orcids":[]},"institutions":[{"id":"https://openalex.org/I99464096","display_name":"KU Leuven","ror":"https://ror.org/05f950310","country_code":"BE","type":"education","lineage":["https://openalex.org/I99464096"]}],"countries":["BE"],"is_corresponding":false,"raw_author_name":"Joost P. Schoeber","raw_affiliation_strings":["Pediatric Nephrology, Katholieke Universiteit Leuven, Herestraat 49 Box 817, Leuven, Belgium"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"Pediatric Nephrology, Katholieke Universiteit Leuven, Herestraat 49 Box 817, Leuven, Belgium","institution_ids":["https://openalex.org/I99464096"]}]},{"author_position":"middle","author":{"id":"https://openalex.org/A5031229398","display_name":"Dorien Reijnders","orcid":"https://orcid.org/0000-0002-6637-4553","observed_orcids":["https://orcid.org/0000-0002-6637-4553"]},"institutions":[{"id":"https://openalex.org/I145872427","display_name":"Radboud University Nijmegen","ror":"https://ror.org/016xsfp80","country_code":"NL","type":"education","lineage":["https://openalex.org/I145872427"]},{"id":"https://openalex.org/I2802934949","display_name":"Radboud University Medical Center","ror":"https://ror.org/05wg1m734","country_code":"NL","type":"funder","lineage":["https://openalex.org/I2802934949"]}],"countries":["NL"],"is_corresponding":false,"raw_author_name":"Dorien Reijnders","raw_affiliation_strings":["Department of Pediatrics, Radboud University Nijmegen Medical Center, Geert Grooteplein Zuid 10, 6525 GA, Nijmegen, The Netherlands"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"Department of Pediatrics, Radboud University Nijmegen Medical Center, Geert Grooteplein Zuid 10, 6525 GA, Nijmegen, The Netherlands","institution_ids":["https://openalex.org/I145872427","https://openalex.org/I2802934949"]}]},{"author_position":"middle","author":{"id":"https://openalex.org/A5003287268","display_name":"Lambertus P. van den Heuvel","orcid":"https://orcid.org/0000-0003-3917-6727","observed_orcids":["https://orcid.org/0000-0003-3917-6727"]},"institutions":[{"id":"https://openalex.org/I145872427","display_name":"Radboud University Nijmegen","ror":"https://ror.org/016xsfp80","country_code":"NL","type":"education","lineage":["https://openalex.org/I145872427"]},{"id":"https://openalex.org/I2802934949","display_name":"Radboud University Medical Center","ror":"https://ror.org/05wg1m734","country_code":"NL","type":"funder","lineage":["https://openalex.org/I2802934949"]},{"id":"https://openalex.org/I99464096","display_name":"KU Leuven","ror":"https://ror.org/05f950310","country_code":"BE","type":"education","lineage":["https://openalex.org/I99464096"]}],"countries":["BE","NL"],"is_corresponding":false,"raw_author_name":"Lambertus P. van den Heuvel","raw_affiliation_strings":["Department of Pediatrics, Radboud University Nijmegen Medical Center, Geert Grooteplein Zuid 10, 6525 GA, Nijmegen, The Netherlands","Pediatric Nephrology, Katholieke Universiteit Leuven, Herestraat 49 Box 817, Leuven, Belgium"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"Department of Pediatrics, Radboud University Nijmegen Medical Center, Geert Grooteplein Zuid 10, 6525 GA, Nijmegen, The Netherlands","institution_ids":["https://openalex.org/I145872427","https://openalex.org/I2802934949"]},{"raw_affiliation_string":"Pediatric Nephrology, Katholieke Universiteit Leuven, Herestraat 49 Box 817, Leuven, Belgium","institution_ids":["https://openalex.org/I99464096"]}]},{"author_position":"middle","author":{"id":"https://openalex.org/A5047818086","display_name":"Rosalinde Masereeuw","orcid":"https://orcid.org/0000-0002-1560-1074","observed_orcids":["https://orcid.org/0000-0002-1560-1074"]},"institutions":[{"id":"https://openalex.org/I145872427","display_name":"Radboud University Nijmegen","ror":"https://ror.org/016xsfp80","country_code":"NL","type":"education","lineage":["https://openalex.org/I145872427"]},{"id":"https://openalex.org/I2802934949","display_name":"Radboud University Medical Center","ror":"https://ror.org/05wg1m734","country_code":"NL","type":"funder","lineage":["https://openalex.org/I2802934949"]}],"countries":["NL"],"is_corresponding":false,"raw_author_name":"Rosalinde Masereeuw","raw_affiliation_strings":["Department of Pharmacology and Toxicology (149), Radboud University Nijmegen Medical Center, Geert Grooteplein Noord 21, 6525 GA, Nijmegen, The Netherlands"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"Department of Pharmacology and Toxicology (149), Radboud University Nijmegen Medical Center, Geert Grooteplein Noord 21, 6525 GA, Nijmegen, The Netherlands","institution_ids":["https://openalex.org/I145872427","https://openalex.org/I2802934949"]}]},{"author_position":"last","author":{"id":"https://openalex.org/A5028156101","display_name":"Elena N. Levtchenko","orcid":"https://orcid.org/0000-0002-8352-7312","observed_orcids":["https://orcid.org/0000-0002-8352-7312"]},"institutions":[{"id":"https://openalex.org/I99464096","display_name":"KU Leuven","ror":"https://ror.org/05f950310","country_code":"BE","type":"education","lineage":["https://openalex.org/I99464096"]}],"countries":["BE"],"is_corresponding":true,"raw_author_name":"Elena Levtchenko","raw_affiliation_strings":["Pediatric Nephrology, Katholieke Universiteit Leuven, Herestraat 49 Box 817, Leuven, Belgium"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"Pediatric Nephrology, Katholieke Universiteit Leuven, Herestraat 49 Box 817, Leuven, Belgium","institution_ids":["https://openalex.org/I99464096"]}]}],"institutions":[],"countries_distinct_count":2,"institutions_distinct_count":3,"corresponding_author_ids":["https://openalex.org/A5028156101"],"corresponding_institution_ids":["https://openalex.org/I99464096"],"apc_list":{"value":2900,"currency":"CHF","value_usd":3499},"apc_paid":{"value":2900,"currency":"CHF","value_usd":3499},"fwci":0.2251,"has_fulltext":true,"cited_by_count":14,"citation_normalized_percentile":{"value":0.63041282,"is_in_top_1_percent":false,"is_in_top_10_percent":false},"cited_by_percentile_year":{"min":89,"max":96},"biblio":{"volume":"3","issue":"4","first_page":"782","last_page":"792"},"is_retracted":false,"is_paratext":false,"is_xpac":false,"primary_topic":{"id":"https://openalex.org/T14415","display_name":"Biomedical Research and Pathophysiology","score":0.9916999936103821,"subfield":{"id":"https://openalex.org/subfields/2734","display_name":"Pathology and Forensic 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/T14415","display_name":"Biomedical Research and Pathophysiology","score":0.9916999936103821,"subfield":{"id":"https://openalex.org/subfields/2734","display_name":"Pathology and Forensic 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/T10570","display_name":"Drug Transport and Resistance Mechanisms","score":0.0032999999821186066,"subfield":{"id":"https://openalex.org/subfields/2730","display_name":"Oncology"},"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/T12770","display_name":"Amino Acid Enzymes and Metabolism","score":0.000699999975040555,"subfield":{"id":"https://openalex.org/subfields/1303","display_name":"Biochemistry"},"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/p-glycoprotein","display_name":"P-glycoprotein","score":0.9760000109672546},{"id":"https://openalex.org/keywords/cystinosis","display_name":"cystinosis","score":0.996999979019165},{"id":"https://openalex.org/keywords/fanconi-syndrome","display_name":"Fanconi syndrome","score":0.8519999980926514},{"id":"https://openalex.org/keywords/proximal-tubular-epithelial-cells","display_name":"proximal tubular epithelial cells","score":0.8820000290870667},{"id":"https://openalex.org/keywords/cysteamine","display_name":"cysteamine","score":0.7879999876022339},{"id":"https://openalex.org/keywords/cystinosin","display_name":"cystinosin","score":0.8190000057220459},{"id":"https://openalex.org/keywords/phosphate-uptake","display_name":"phosphate uptake","score":0.8379999995231628},{"id":"https://openalex.org/keywords/psc-833","display_name":"PSC833","score":0.7200000286102295},{"id":"https://openalex.org/keywords/renal-tubular-transport","display_name":"renal tubular transport","score":0.49900001287460327},{"id":"https://openalex.org/keywords/cystine-accumulation","display_name":"cystine accumulation","score":0.5669999718666077}],"concepts":[{"id":"https://openalex.org/C2778518273","wikidata":"https://www.wikidata.org/wiki/Q1149042","display_name":"Cystinosis","level":5,"score":0.9725837707519531},{"id":"https://openalex.org/C2776609658","wikidata":"https://www.wikidata.org/wiki/Q617563","display_name":"Cysteamine","level":2,"score":0.8729606866836548},{"id":"https://openalex.org/C2779026464","wikidata":"https://www.wikidata.org/wiki/Q1179460","display_name":"Fanconi syndrome","level":3,"score":0.8571466207504272},{"id":"https://openalex.org/C134018914","wikidata":"https://www.wikidata.org/wiki/Q162606","display_name":"Endocrinology","level":1,"score":0.6251728534698486},{"id":"https://openalex.org/C126322002","wikidata":"https://www.wikidata.org/wiki/Q11180","display_name":"Internal medicine","level":1,"score":0.6209695935249329},{"id":"https://openalex.org/C2777608289","wikidata":"https://www.wikidata.org/wiki/Q408626","display_name":"Cystine","level":4,"score":0.5435360670089722},{"id":"https://openalex.org/C2780091579","wikidata":"https://www.wikidata.org/wiki/Q9377","display_name":"Kidney","level":2,"score":0.4926486611366272},{"id":"https://openalex.org/C149011108","wikidata":"https://www.wikidata.org/wiki/Q652985","display_name":"Transporter","level":3,"score":0.48571616411209106},{"id":"https://openalex.org/C2778707650","wikidata":"https://www.wikidata.org/wiki/Q412902","display_name":"P-glycoprotein","level":4,"score":0.4843612611293793},{"id":"https://openalex.org/C185592680","wikidata":"https://www.wikidata.org/wiki/Q2329","display_name":"Chemistry","level":0,"score":0.48190122842788696},{"id":"https://openalex.org/C2778264360","wikidata":"https://www.wikidata.org/wiki/Q675010","display_name":"Lysosomal storage disease","level":3,"score":0.48027849197387695},{"id":"https://openalex.org/C71924100","wikidata":"https://www.wikidata.org/wiki/Q11190","display_name":"Medicine","level":0,"score":0.33919334411621094},{"id":"https://openalex.org/C55493867","wikidata":"https://www.wikidata.org/wiki/Q7094","display_name":"Biochemistry","level":1,"score":0.23509415984153748},{"id":"https://openalex.org/C2779134260","wikidata":"https://www.wikidata.org/wiki/Q12136","display_name":"Disease","level":2,"score":0.12249407172203064},{"id":"https://openalex.org/C181199279","wikidata":"https://www.wikidata.org/wiki/Q8047","display_name":"Enzyme","level":2,"score":0.0738382339477539},{"id":"https://openalex.org/C104317684","wikidata":"https://www.wikidata.org/wiki/Q7187","display_name":"Gene","level":2,"score":0.06977793574333191},{"id":"https://openalex.org/C133936738","wikidata":"https://www.wikidata.org/wiki/Q643839","display_name":"Multiple drug resistance","level":3,"score":0.0},{"id":"https://openalex.org/C2779201268","wikidata":"https://www.wikidata.org/wiki/Q186474","display_name":"Cysteine","level":3,"score":0.0},{"id":"https://openalex.org/C501593827","wikidata":"https://www.wikidata.org/wiki/Q12187","display_name":"Antibiotics","level":2,"score":0.0}],"mesh":[],"locations_count":8,"locations":[{"id":"doi:10.3390/pharmaceutics3040782","doi":"https://doi.org/10.3390/pharmaceutics3040782","is_oa":true,"landing_page_url":"https://doi.org/10.3390/pharmaceutics3040782","pdf_url":"https://www.mdpi.com/1999-4923/3/4/782/pdf?version=1319718870","source":{"id":"https://openalex.org/S30283931","display_name":"Pharmaceutics","issn_l":"1999-4923","issn":["1999-4923"],"is_oa":true,"is_in_doaj":true,"is_core":true,"listed_in":["cwts-core","doaj","norway-1","poland-100","russia-white-list-1","vabb-shw"],"host_organization":"https://openalex.org/P4310310987","host_organization_name":"Multidisciplinary Digital Publishing Institute","host_organization_lineage":["https://openalex.org/P4310310987"],"host_organization_lineage_names":["Multidisciplinary Digital Publishing Institute"],"type":"journal"},"license":"cc-by","license_id":"https://openalex.org/licenses/cc-by","version":"publishedVersion","is_accepted":true,"is_published":true,"raw_source_name":"Pharmaceutics","raw_type":"journal-article"},{"id":"pmh:oai:europepmc.org:2847899","doi":null,"is_oa":true,"landing_page_url":"https://www.ncbi.nlm.nih.gov/pmc/articles/3857058","pdf_url":null,"source":{"id":"https://openalex.org/S4306400806","display_name":"Europe PMC (PubMed Central)","issn_l":null,"issn":null,"is_oa":false,"is_in_doaj":false,"is_core":false,"listed_in":[],"host_organization":"https://openalex.org/I1303153112","host_organization_name":"European Bioinformatics Institute","host_organization_lineage":["https://openalex.org/I1303153112"],"host_organization_lineage_names":[],"type":"repository"},"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":"Text"},{"id":"pmh:oai:pubmedcentral.nih.gov:3857058","doi":null,"is_oa":true,"landing_page_url":"https://www.ncbi.nlm.nih.gov/pmc/articles/3857058","pdf_url":null,"source":{"id":"https://openalex.org/S2764455111","display_name":"PubMed Central","issn_l":null,"issn":null,"is_oa":true,"is_in_doaj":false,"is_core":false,"listed_in":[],"host_organization":"https://openalex.org/I1299303238","host_organization_name":"National Institutes of Health","host_organization_lineage":["https://openalex.org/I1299303238"],"host_organization_lineage_names":[],"type":"repository"},"license":"cc-by","license_id":"https://openalex.org/licenses/cc-by","version":"publishedVersion","is_accepted":true,"is_published":true,"raw_source_name":"Pharmaceutics","raw_type":"Text"},{"id":"pmid:24309308","doi":null,"is_oa":false,"landing_page_url":"https://pubmed.ncbi.nlm.nih.gov/24309308","pdf_url":null,"source":{"id":"https://openalex.org/S4306525036","display_name":"PubMed","issn_l":null,"issn":null,"is_oa":false,"is_in_doaj":false,"is_core":false,"listed_in":[],"host_organization":"https://openalex.org/I1299303238","host_organization_name":"National Institutes of Health","host_organization_lineage":["https://openalex.org/I1299303238"],"host_organization_lineage_names":[],"type":"repository"},"license":null,"license_id":null,"version":"publishedVersion","is_accepted":true,"is_published":true,"raw_source_name":"Pharmaceutics","raw_type":"Journal Article"},{"id":"pmh:oai:lirias2repo.kuleuven.be:123456789/653719","doi":null,"is_oa":true,"landing_page_url":"https://lirias.kuleuven.be/handle/123456789/653719","pdf_url":null,"source":{"id":"https://openalex.org/S7407055369","display_name":"Lirias","issn_l":null,"issn":null,"is_oa":false,"is_in_doaj":false,"is_core":false,"listed_in":[],"host_organization":null,"host_organization_name":null,"host_organization_lineage":[],"host_organization_lineage_names":[],"type":"repository"},"license":"other-oa","license_id":"https://openalex.org/licenses/other-oa","version":"acceptedVersion","is_accepted":true,"is_published":false,"raw_source_name":"Pharmaceutics, vol. 3 (4), (782-792)","raw_type":"info:eu-repo/semantics/article"},{"id":"pmh:ru:oai:repository.ubn.ru.nl:2066/96306","doi":null,"is_oa":true,"landing_page_url":"http://hdl.handle.net/2066/96306","pdf_url":"http://hdl.handle.net/2066/96306","source":{"id":"https://openalex.org/S4306401067","display_name":"Radboud Repository (Radboud University)","issn_l":null,"issn":null,"is_oa":false,"is_in_doaj":false,"is_core":false,"listed_in":[],"host_organization":"https://openalex.org/I145872427","host_organization_name":"Radboud University Nijmegen","host_organization_lineage":["https://openalex.org/I145872427"],"host_organization_lineage_names":[],"type":"repository"},"license":"other-oa","license_id":"https://openalex.org/licenses/other-oa","version":"submittedVersion","is_accepted":false,"is_published":false,"raw_source_name":"Pharmaceutics, 3, 782 - 92","raw_type":"info:eu-repo/semantics/article"},{"id":"pmh:oai:doaj.org/article:88f1588550094a7782e6279e180e8963","doi":null,"is_oa":true,"landing_page_url":"https://doaj.org/article/88f1588550094a7782e6279e180e8963","pdf_url":null,"source":{"id":"https://openalex.org/S4306401280","display_name":"DOAJ (DOAJ: Directory of Open Access Journals)","issn_l":null,"issn":null,"is_oa":false,"is_in_doaj":false,"is_core":false,"listed_in":[],"host_organization":null,"host_organization_name":null,"host_organization_lineage":[],"host_organization_lineage_names":[],"type":"repository"},"license":"cc-by-sa","license_id":"https://openalex.org/licenses/cc-by-sa","version":"submittedVersion","is_accepted":false,"is_published":false,"raw_source_name":"Pharmaceutics, Vol 3, Iss 4, Pp 782-792 (2011)","raw_type":"article"},{"id":"pmh:oai:repository.ubn.ru.nl:2066/96306","doi":null,"is_oa":false,"landing_page_url":"http://hdl.handle.net/2066/96306","pdf_url":null,"source":{"id":"https://openalex.org/S4306401067","display_name":"Radboud Repository (Radboud University)","issn_l":null,"issn":null,"is_oa":false,"is_in_doaj":false,"is_core":false,"listed_in":[],"host_organization":"https://openalex.org/I145872427","host_organization_name":"Radboud University Nijmegen","host_organization_lineage":["https://openalex.org/I145872427"],"host_organization_lineage_names":[],"type":"repository"},"license":null,"license_id":null,"version":"submittedVersion","is_accepted":false,"is_published":false,"raw_source_name":"","raw_type":"Article / Letter to editor"}],"best_oa_location":{"id":"doi:10.3390/pharmaceutics3040782","doi":"https://doi.org/10.3390/pharmaceutics3040782","is_oa":true,"landing_page_url":"https://doi.org/10.3390/pharmaceutics3040782","pdf_url":"https://www.mdpi.com/1999-4923/3/4/782/pdf?version=1319718870","source":{"id":"https://openalex.org/S30283931","display_name":"Pharmaceutics","issn_l":"1999-4923","issn":["1999-4923"],"is_oa":true,"is_in_doaj":true,"is_core":true,"listed_in":["cwts-core","doaj","norway-1","poland-100","russia-white-list-1","vabb-shw"],"host_organization":"https://openalex.org/P4310310987","host_organization_name":"Multidisciplinary Digital Publishing Institute","host_organization_lineage":["https://openalex.org/P4310310987"],"host_organization_lineage_names":["Multidisciplinary Digital Publishing Institute"],"type":"journal"},"license":"cc-by","license_id":"https://openalex.org/licenses/cc-by","version":"publishedVersion","is_accepted":true,"is_published":true,"raw_source_name":"Pharmaceutics","raw_type":"journal-article"},"sustainable_development_goals":[{"id":"https://metadata.un.org/sdg/3","score":0.9537000060081482,"display_name":"Good health and well-being"}],"study_designs":[],"awards":[{"id":"https://openalex.org/G5569153993","display_name":"European Network for the Study of Orphan Nephropathies","funder_award_id":"201590","funder_id":"https://openalex.org/F4320320300","funder_display_name":"European Commission"}],"funders":[{"id":"https://openalex.org/F4320320300","display_name":"European Commission","ror":"https://ror.org/00k4n6c32"}],"has_content":{"pdf":true,"grobid_xml":true},"content_urls":{"pdf":"https://content.openalex.org/works/W2158024088.pdf","grobid_xml":"https://content.openalex.org/works/W2158024088.grobid-xml"},"referenced_works_count":15,"referenced_works":["https://openalex.org/W1967287259","https://openalex.org/W1985266383","https://openalex.org/W1999363531","https://openalex.org/W2012589757","https://openalex.org/W2026574583","https://openalex.org/W2029870086","https://openalex.org/W2043114811","https://openalex.org/W2069791449","https://openalex.org/W2083279563","https://openalex.org/W2130307549","https://openalex.org/W2130825005","https://openalex.org/W2136000553","https://openalex.org/W2144317785","https://openalex.org/W2171320890","https://openalex.org/W2413507536"],"related_works":["https://openalex.org/W7100446519","https://openalex.org/W2524702367","https://openalex.org/W1986958627","https://openalex.org/W2071398645","https://openalex.org/W2079766707","https://openalex.org/W7101138581","https://openalex.org/W1964819412","https://openalex.org/W2084949442","https://openalex.org/W1983980368","https://openalex.org/W4407429928"],"abstract_inverted_index":{"P-glycoprotein":[0],"(P-gp)":[1],"is":[2,73,127,221,250],"an":[3,56],"ATP-dependent":[4],"transporter":[5,67,125],"localized":[6],"at":[7],"the":[8,12,21,50,64,79,100,104,113,121,137,166,171,176,191,224,254],"apical":[9,80],"membrane":[10],"of":[11,23,52,78,106,139,175,193,214,226,256],"kidney":[13],"proximal":[14,45,81,122,148,215],"tubules,":[15],"which":[16],"plays":[17],"a":[18],"role":[19],"in":[20,38,41,63,71,103,129,170,183,208,230,243,253],"efflux":[22,124],"cationic":[24],"and":[25,30,92,131,157,173,199,211,239],"amphipathic":[26],"endogenous":[27],"waste":[28],"products":[29],"xenobiotics,":[31],"such":[32],"as":[33,186],"drugs,":[34],"into":[35],"urine.":[36],"Studies":[37],"mice":[39],"deficient":[40],"P-gp":[42,126,167,179,196,206,237,248],"showed":[43],"generalized":[44,76],"tubular":[46,82,123,149,216],"dysfunction":[47,77,249],"similar":[48],"to":[49,136,188],"phenotype":[51],"patients":[53,107,156],"with":[54,112,223],"cystinosis,":[55],"autosomal":[57],"recessive":[58],"disorder":[59],"caused":[60],"by":[61,75,164],"mutations":[62],"lysosomal":[65],"cystine":[66],"cystinosin.":[68],"Renal":[69],"disease":[70,98],"cystinosis":[72,130],"characterized":[74],"influx":[83],"transporters":[84],"(so-called":[85],"renal":[86,97,140,227],"Fanconi":[87,141,228],"syndrome)":[88],"developing":[89],"during":[90],"infancy":[91],"gradually":[93],"progressing":[94],"towards":[95],"end-stage":[96],"before":[99],"10th":[101],"birthday":[102],"majority":[105],"that":[108,247],"are":[109,241],"not":[110,251],"treated":[111],"cystine-depleting":[114],"drug":[115],"cysteamine.":[116],"Here,":[117],"we":[118],"investigated":[119],"whether":[120,132],"affected":[128],"this":[133],"might":[134],"contribute":[135],"development":[138],"syndrome.":[142],"We":[143],"used":[144],"conditionally":[145],"immortalized":[146],"(ci)":[147],"epithelial":[150],"cells":[151,185],"(ciPTEC)":[152],"derived":[153],"from":[154],"cystinotic":[155,184,209,244],"healthy":[158],"volunteers.":[159],"P-gp-mediated":[160],"transport":[161,197],"was":[162,181,202],"measured":[163],"using":[165],"substrate":[168],"calcein-AM":[169],"presence":[172],"absence":[174],"P-gp-inhibitor":[177],"PSC833.":[178],"activity":[180,198,207,240],"normal":[182,242],"compared":[187],"controls.":[189],"Additionally,":[190],"effect":[192],"cysteamine":[194,233],"on":[195],"phosphate":[200,217],"uptake":[201],"determined;":[203],"demonstrating":[204],"increased":[205],"cells,":[210],"further":[212],"decrease":[213],"uptake.":[218],"This":[219],"observation":[220],"compatible":[222],"persistence":[225],"syndrome":[229],"vivo":[231],"under":[232],"therapy.":[234],"In":[235],"summary,":[236],"expression":[238],"ciPTEC,":[245],"indicating":[246],"involved":[252],"pathogenesis":[255],"cystinosis.":[257]},"counts_by_year":[{"year":2026,"cited_by_count":1},{"year":2024,"cited_by_count":1},{"year":2023,"cited_by_count":2},{"year":2022,"cited_by_count":2},{"year":2021,"cited_by_count":3},{"year":2020,"cited_by_count":1},{"year":2019,"cited_by_count":1},{"year":2015,"cited_by_count":2},{"year":2012,"cited_by_count":1}],"updated_date":"2026-10-28T10:33:24.094632","created_date":"2016-06-24T00:00:00"}