{"id":"https://openalex.org/W2312957424","doi":"https://doi.org/10.3727/096368913x666980","title":"Hepatocyte Transplantation Ameliorates the Metabolic Abnormality in a Mouse Model of Acute Intermittent Porphyria","display_name":"Hepatocyte Transplantation Ameliorates the Metabolic Abnormality in a Mouse Model of Acute Intermittent Porphyria","publication_year":2013,"publication_date":"2013-04-15","ids":{"openalex":"https://openalex.org/W2312957424","doi":"https://doi.org/10.3727/096368913x666980","mag":"2312957424","pmid":"https://pubmed.ncbi.nlm.nih.gov/23582197"},"language":"en","primary_location":{"id":"doi:10.3727/096368913x666980","doi":"https://doi.org/10.3727/096368913x666980","is_oa":false,"landing_page_url":"https://doi.org/10.3727/096368913x666980","pdf_url":null,"source":{"id":"https://openalex.org/S165333527","display_name":"Cell Transplantation","issn_l":"0963-6897","issn":["0963-6897","1555-3892"],"is_oa":false,"is_in_doaj":true,"is_core":true,"listed_in":["cwts-core","doaj","dongbi-c","doyens","jufo-1","ki-jl-1","medline","norway-1","oarl","poland-70","russia-white-list-1","vabb-shw"],"host_organization":"https://openalex.org/P4310320017","host_organization_name":"SAGE Publishing","host_organization_lineage":["https://openalex.org/P4310320017"],"host_organization_lineage_names":["SAGE Publishing"],"type":"journal"},"license":null,"license_id":null,"version":"publishedVersion","is_accepted":true,"is_published":true,"raw_source_name":"Cell Transplantation","raw_type":"journal-article"},"volume":null,"type":"article","indexed_in":["crossref","doaj","pubmed"],"open_access":{"is_oa":true,"oa_status":"green","oa_url":"https://doaj.org/article/830696ab05e04207b0d204023e67ea05","any_repository_has_fulltext":true},"authorships":[{"author_position":"first","author":{"id":null,"display_name":"Zhaohui Yin","orcid":null,"observed_orcids":[]},"institutions":[{"id":"https://openalex.org/I1335113835","display_name":"Karolinska University Hospital","ror":"https://ror.org/00m8d6786","country_code":"SE","type":"healthcare","lineage":["https://openalex.org/I1335113835"]},{"id":"https://openalex.org/I4210115239","display_name":"First Affiliated Hospital of Shantou University Medical College","ror":"https://ror.org/02bnz8785","country_code":"CN","type":"healthcare","lineage":["https://openalex.org/I4210115239"]},{"id":"https://openalex.org/I4210120522","display_name":"Shantou University Medical College","ror":"https://ror.org/02gxych78","country_code":"CN","type":"education","lineage":["https://openalex.org/I4210120522"]}],"countries":["CN","SE"],"is_corresponding":false,"raw_author_name":"Zhaohui Yin","raw_affiliation_strings":["Department of General Surgery, First Hospital of Shantou University Medical College, Shantou, Guangdong Province, China","Division of Transplantation Surgery, Department for Clinical Science, Intervention and Technology CLINTEC, Karolinska University Hospital, Stockholm, Sweden"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"Department of General Surgery, First Hospital of Shantou University Medical College, Shantou, Guangdong Province, China","institution_ids":["https://openalex.org/I4210115239","https://openalex.org/I4210120522"]},{"raw_affiliation_string":"Division of Transplantation Surgery, Department for Clinical Science, Intervention and Technology CLINTEC, Karolinska University Hospital, Stockholm, Sweden","institution_ids":["https://openalex.org/I1335113835"]}]},{"author_position":"middle","author":{"id":"https://openalex.org/A5076343477","display_name":"Staffan W\u00e5hlin","orcid":"https://orcid.org/0000-0002-5985-9464","observed_orcids":["https://orcid.org/0000-0002-5985-9464"]},"institutions":[{"id":"https://openalex.org/I1335113835","display_name":"Karolinska University Hospital","ror":"https://ror.org/00m8d6786","country_code":"SE","type":"healthcare","lineage":["https://openalex.org/I1335113835"]},{"id":"https://openalex.org/I28166907","display_name":"Karolinska Institutet","ror":"https://ror.org/056d84691","country_code":"SE","type":"education","lineage":["https://openalex.org/I28166907"]}],"countries":["SE"],"is_corresponding":true,"raw_author_name":"Staffan Wahlin","raw_affiliation_strings":["Department of Gastroenterology and Hepatology, Karolinska Institutet, Karolinska University Hospital, Stockholm, Sweden"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"Department of Gastroenterology and Hepatology, Karolinska Institutet, Karolinska University Hospital, Stockholm, Sweden","institution_ids":["https://openalex.org/I1335113835","https://openalex.org/I28166907"]}]},{"author_position":"middle","author":{"id":"https://openalex.org/A5085737685","display_name":"Ewa C. S. Ellis","orcid":"https://orcid.org/0000-0002-3057-5337","observed_orcids":["https://orcid.org/0000-0002-3057-5337"]},"institutions":[{"id":"https://openalex.org/I1335113835","display_name":"Karolinska University Hospital","ror":"https://ror.org/00m8d6786","country_code":"SE","type":"healthcare","lineage":["https://openalex.org/I1335113835"]}],"countries":["SE"],"is_corresponding":false,"raw_author_name":"Ewa C. S. Ellis","raw_affiliation_strings":["Division of Transplantation Surgery, Department for Clinical Science, Intervention and Technology CLINTEC, Karolinska University Hospital, Stockholm, Sweden"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"Division of Transplantation Surgery, Department for Clinical Science, Intervention and Technology CLINTEC, Karolinska University Hospital, Stockholm, Sweden","institution_ids":["https://openalex.org/I1335113835"]}]},{"author_position":"middle","author":{"id":"https://openalex.org/A5007837022","display_name":"Pauline Harper","orcid":"https://orcid.org/0000-0001-7336-2801","observed_orcids":["https://orcid.org/0000-0001-7336-2801"]},"institutions":[{"id":"https://openalex.org/I1335113835","display_name":"Karolinska University Hospital","ror":"https://ror.org/00m8d6786","country_code":"SE","type":"healthcare","lineage":["https://openalex.org/I1335113835"]}],"countries":["SE"],"is_corresponding":false,"raw_author_name":"Pauline Harper","raw_affiliation_strings":["Porphyria Centre Sweden, Division of Metabolic Diseases, Karolinska University Hospital, Stockholm, Sweden"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"Porphyria Centre Sweden, Division of Metabolic Diseases, Karolinska University Hospital, Stockholm, Sweden","institution_ids":["https://openalex.org/I1335113835"]}]},{"author_position":"middle","author":{"id":"https://openalex.org/A5012898633","display_name":"Bo\u2010G\u00f6ran Ericzon","orcid":"https://orcid.org/0000-0001-5604-2427","observed_orcids":["https://orcid.org/0000-0001-5604-2427"]},"institutions":[{"id":"https://openalex.org/I1335113835","display_name":"Karolinska University Hospital","ror":"https://ror.org/00m8d6786","country_code":"SE","type":"healthcare","lineage":["https://openalex.org/I1335113835"]}],"countries":["SE"],"is_corresponding":false,"raw_author_name":"Bo-G\u00f6ran Ericzon","raw_affiliation_strings":["Division of Transplantation Surgery, Department for Clinical Science, Intervention and Technology CLINTEC, Karolinska University Hospital, Stockholm, Sweden"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"Division of Transplantation Surgery, Department for Clinical Science, Intervention and Technology CLINTEC, Karolinska University Hospital, Stockholm, Sweden","institution_ids":["https://openalex.org/I1335113835"]}]},{"author_position":"last","author":{"id":"https://openalex.org/A5066684311","display_name":"Greg Nowak","orcid":"https://orcid.org/0000-0001-8766-8860","observed_orcids":["https://orcid.org/0000-0001-8766-8860"]},"institutions":[{"id":"https://openalex.org/I1335113835","display_name":"Karolinska University Hospital","ror":"https://ror.org/00m8d6786","country_code":"SE","type":"healthcare","lineage":["https://openalex.org/I1335113835"]}],"countries":["SE"],"is_corresponding":false,"raw_author_name":"Greg Nowak","raw_affiliation_strings":["Division of Transplantation Surgery, Department for Clinical Science, Intervention and Technology CLINTEC, Karolinska University Hospital, Stockholm, Sweden"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"Division of Transplantation Surgery, Department for Clinical Science, Intervention and Technology CLINTEC, Karolinska University Hospital, Stockholm, Sweden","institution_ids":["https://openalex.org/I1335113835"]}]}],"institutions":[],"countries_distinct_count":2,"institutions_distinct_count":4,"corresponding_author_ids":["https://openalex.org/A5076343477"],"corresponding_institution_ids":["https://openalex.org/I1335113835","https://openalex.org/I28166907"],"apc_list":{"value":2650,"currency":"USD","value_usd":2650},"apc_paid":null,"fwci":0.8789,"has_fulltext":false,"cited_by_count":20,"citation_normalized_percentile":{"value":0.72198998,"is_in_top_1_percent":false,"is_in_top_10_percent":false},"cited_by_percentile_year":{"min":89,"max":98},"biblio":{"volume":"23","issue":"9","first_page":"1153","last_page":"1162"},"is_retracted":false,"is_paratext":false,"is_xpac":false,"primary_topic":{"id":"https://openalex.org/T12441","display_name":"Porphyrin Metabolism and Disorders","score":0.9990000128746033,"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"}},"topics":[{"id":"https://openalex.org/T12441","display_name":"Porphyrin Metabolism and Disorders","score":0.9990000128746033,"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/T11027","display_name":"Metabolism and Genetic Disorders","score":0.00019999999494757503,"subfield":{"id":"https://openalex.org/subfields/1308","display_name":"Clinical 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"}},{"id":"https://openalex.org/T12068","display_name":"Neonatal Health and Biochemistry","score":9.999999747378752e-05,"subfield":{"id":"https://openalex.org/subfields/2735","display_name":"Pediatrics, Perinatology and Child Health"},"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/acute-intermittent-porphyria","display_name":"acute intermittent porphyria","score":0.9940000176429749},{"id":"https://openalex.org/keywords/hepatocyte-transplantation","display_name":"hepatocyte transplantation","score":0.9819999933242798},{"id":"https://openalex.org/keywords/porphobilinogen-deaminase-deficiency","display_name":"porphobilinogen deaminase deficiency","score":0.9470000267028809},{"id":"https://openalex.org/keywords/heme-biosynthesis","display_name":"heme biosynthesis","score":0.7099999785423279},{"id":"https://openalex.org/keywords/alas1","display_name":"ALAS1","score":0.7919999957084656},{"id":"https://openalex.org/keywords/cyp2a5","display_name":"CYP2A5","score":0.9890000224113464},{"id":"https://openalex.org/keywords/hepatocyte-engraftment","display_name":"hepatocyte engraftment","score":0.8479999899864197},{"id":"https://openalex.org/keywords/mouse-model","display_name":"mouse model","score":0.6029999852180481}],"concepts":[{"id":"https://openalex.org/C2778503633","wikidata":"https://www.wikidata.org/wiki/Q415227","display_name":"Porphobilinogen deaminase","level":4,"score":0.9446814060211182},{"id":"https://openalex.org/C2779028619","wikidata":"https://www.wikidata.org/wiki/Q424247","display_name":"Acute intermittent porphyria","level":3,"score":0.8617638349533081},{"id":"https://openalex.org/C2776682811","wikidata":"https://www.wikidata.org/wiki/Q421178","display_name":"Porphobilinogen","level":3,"score":0.7753753066062927},{"id":"https://openalex.org/C2776200302","wikidata":"https://www.wikidata.org/wiki/Q827450","display_name":"Hepatocyte","level":3,"score":0.7045956254005432},{"id":"https://openalex.org/C2911091166","wikidata":"https://www.wikidata.org/wiki/Q106419912","display_name":"Transplantation","level":2,"score":0.6440211534500122},{"id":"https://openalex.org/C2777940757","wikidata":"https://www.wikidata.org/wiki/Q271759","display_name":"Porphyria","level":2,"score":0.557168185710907},{"id":"https://openalex.org/C2776217839","wikidata":"https://www.wikidata.org/wiki/Q189621","display_name":"Heme","level":3,"score":0.5347261428833008},{"id":"https://openalex.org/C44538786","wikidata":"https://www.wikidata.org/wiki/Q17816567","display_name":"Porphobilinogen synthase","level":4,"score":0.4836314916610718},{"id":"https://openalex.org/C126322002","wikidata":"https://www.wikidata.org/wiki/Q11180","display_name":"Internal medicine","level":1,"score":0.48239147663116455},{"id":"https://openalex.org/C86803240","wikidata":"https://www.wikidata.org/wiki/Q420","display_name":"Biology","level":0,"score":0.4629104733467102},{"id":"https://openalex.org/C134018914","wikidata":"https://www.wikidata.org/wiki/Q162606","display_name":"Endocrinology","level":1,"score":0.4401331841945648},{"id":"https://openalex.org/C50965678","wikidata":"https://www.wikidata.org/wiki/Q2724302","display_name":"Abnormality","level":2,"score":0.4233753979206085},{"id":"https://openalex.org/C181199279","wikidata":"https://www.wikidata.org/wiki/Q8047","display_name":"Enzyme","level":2,"score":0.382088303565979},{"id":"https://openalex.org/C185592680","wikidata":"https://www.wikidata.org/wiki/Q2329","display_name":"Chemistry","level":0,"score":0.33950936794281006},{"id":"https://openalex.org/C55493867","wikidata":"https://www.wikidata.org/wiki/Q7094","display_name":"Biochemistry","level":1,"score":0.2495247721672058},{"id":"https://openalex.org/C71924100","wikidata":"https://www.wikidata.org/wiki/Q11190","display_name":"Medicine","level":0,"score":0.22357484698295593},{"id":"https://openalex.org/C202751555","wikidata":"https://www.wikidata.org/wiki/Q221681","display_name":"In vitro","level":2,"score":0.095481276512146},{"id":"https://openalex.org/C118552586","wikidata":"https://www.wikidata.org/wiki/Q7867","display_name":"Psychiatry","level":1,"score":0.0},{"id":"https://openalex.org/C2781374117","wikidata":"https://www.wikidata.org/wiki/Q5252382","display_name":"Dehydratase","level":3,"score":0.0}],"mesh":[{"descriptor_ui":"D000072467","descriptor_name":"Cytochrome P450 Family 2","qualifier_ui":null,"qualifier_name":null,"is_major_topic":false},{"descriptor_ui":"D000622","descriptor_name":"Aminolevulinic Acid","qualifier_ui":"Q000097","qualifier_name":"blood","is_major_topic":false},{"descriptor_ui":"D000624","descriptor_name":"5-Aminolevulinate Synthetase","qualifier_ui":"Q000235","qualifier_name":"genetics","is_major_topic":false},{"descriptor_ui":"D000624","descriptor_name":"5-Aminolevulinate Synthetase","qualifier_ui":"Q000378","qualifier_name":"metabolism","is_major_topic":false},{"descriptor_ui":"D000818","descriptor_name":"Animals","qualifier_ui":null,"qualifier_name":null,"is_major_topic":false},{"descriptor_ui":"D001189","descriptor_name":"Aryl Hydrocarbon Hydroxylases","qualifier_ui":"Q000235","qualifier_name":"genetics","is_major_topic":false},{"descriptor_ui":"D001189","descriptor_name":"Aryl Hydrocarbon Hydroxylases","qualifier_ui":"Q000378","qualifier_name":"metabolism","is_major_topic":false},{"descriptor_ui":"D002478","descriptor_name":"Cells, Cultured","qualifier_ui":null,"qualifier_name":null,"is_major_topic":false},{"descriptor_ui":"D004195","descriptor_name":"Disease Models, Animal","qualifier_ui":null,"qualifier_name":null,"is_major_topic":false},{"descriptor_ui":"D008297","descriptor_name":"Male","qualifier_ui":null,"qualifier_name":null,"is_major_topic":false},{"descriptor_ui":"D008810","descriptor_name":"Mice, Inbred C57BL","qualifier_ui":null,"qualifier_name":null,"is_major_topic":false},{"descriptor_ui":"D011162","descriptor_name":"Porphobilinogen","qualifier_ui":"Q000097","qualifier_name":"blood","is_major_topic":false},{"descriptor_ui":"D011163","descriptor_name":"Hydroxymethylbilane Synthase","qualifier_ui":"Q000235","qualifier_name":"genetics","is_major_topic":false},{"descriptor_ui":"D011163","descriptor_name":"Hydroxymethylbilane Synthase","qualifier_ui":"Q000378","qualifier_name":"metabolism","is_major_topic":false},{"descriptor_ui":"D012333","descriptor_name":"RNA, Messenger","qualifier_ui":"Q000378","qualifier_name":"metabolism","is_major_topic":false},{"descriptor_ui":"D014184","descriptor_name":"Transplantation, Homologous","qualifier_ui":null,"qualifier_name":null,"is_major_topic":false},{"descriptor_ui":"D017118","descriptor_name":"Porphyria, Acute Intermittent","qualifier_ui":"Q000628","qualifier_name":"therapy","is_major_topic":true},{"descriptor_ui":"D022781","descriptor_name":"Hepatocytes","qualifier_ui":"Q000166","qualifier_name":"cytology","is_major_topic":false},{"descriptor_ui":"D022781","descriptor_name":"Hepatocytes","qualifier_ui":"Q000378","qualifier_name":"metabolism","is_major_topic":false},{"descriptor_ui":"D022781","descriptor_name":"Hepatocytes","qualifier_ui":"Q000637","qualifier_name":"transplantation","is_major_topic":true},{"descriptor_ui":"D051379","descriptor_name":"Mice","qualifier_ui":null,"qualifier_name":null,"is_major_topic":false}],"locations_count":3,"locations":[{"id":"doi:10.3727/096368913x666980","doi":"https://doi.org/10.3727/096368913x666980","is_oa":false,"landing_page_url":"https://doi.org/10.3727/096368913x666980","pdf_url":null,"source":{"id":"https://openalex.org/S165333527","display_name":"Cell Transplantation","issn_l":"0963-6897","issn":["0963-6897","1555-3892"],"is_oa":false,"is_in_doaj":true,"is_core":true,"listed_in":["cwts-core","doaj","dongbi-c","doyens","jufo-1","ki-jl-1","medline","norway-1","oarl","poland-70","russia-white-list-1","vabb-shw"],"host_organization":"https://openalex.org/P4310320017","host_organization_name":"SAGE Publishing","host_organization_lineage":["https://openalex.org/P4310320017"],"host_organization_lineage_names":["SAGE Publishing"],"type":"journal"},"license":null,"license_id":null,"version":"publishedVersion","is_accepted":true,"is_published":true,"raw_source_name":"Cell Transplantation","raw_type":"journal-article"},{"id":"pmid:23582197","doi":null,"is_oa":false,"landing_page_url":"https://pubmed.ncbi.nlm.nih.gov/23582197","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":"Cell transplantation","raw_type":"Journal Article"},{"id":"pmh:oai:doaj.org/article:830696ab05e04207b0d204023e67ea05","doi":null,"is_oa":true,"landing_page_url":"https://doaj.org/article/830696ab05e04207b0d204023e67ea05","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":"Cell Transplantation, Vol 23 (2014)","raw_type":"article"}],"best_oa_location":{"id":"pmh:oai:doaj.org/article:830696ab05e04207b0d204023e67ea05","doi":null,"is_oa":true,"landing_page_url":"https://doaj.org/article/830696ab05e04207b0d204023e67ea05","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":"Cell Transplantation, Vol 23 (2014)","raw_type":"article"},"sustainable_development_goals":[{"display_name":"Good health and well-being","id":"https://metadata.un.org/sdg/3","score":0.9940000176429749}],"study_designs":[],"awards":[],"funders":[],"has_content":{"grobid_xml":false,"pdf":false},"content_urls":null,"referenced_works_count":24,"referenced_works":["https://openalex.org/W30013491","https://openalex.org/W89320799","https://openalex.org/W1503993161","https://openalex.org/W1558705752","https://openalex.org/W1903214678","https://openalex.org/W1967089959","https://openalex.org/W1977058448","https://openalex.org/W1979686921","https://openalex.org/W1979721156","https://openalex.org/W1989999391","https://openalex.org/W1996409582","https://openalex.org/W2023877835","https://openalex.org/W2031067362","https://openalex.org/W2033901013","https://openalex.org/W2034973225","https://openalex.org/W2059924653","https://openalex.org/W2064840115","https://openalex.org/W2094863596","https://openalex.org/W2132777822","https://openalex.org/W2133533194","https://openalex.org/W2247149472","https://openalex.org/W2283686865","https://openalex.org/W2403493512","https://openalex.org/W3144847119"],"related_works":["https://openalex.org/W2156152832","https://openalex.org/W4296524037","https://openalex.org/W4388730608","https://openalex.org/W2059924653","https://openalex.org/W2997955340","https://openalex.org/W98639535","https://openalex.org/W2159500294","https://openalex.org/W1974504902","https://openalex.org/W4205428477","https://openalex.org/W4403128580"],"abstract_inverted_index":{"Acute":[0],"intermittent":[1],"porphyria":[2],"(AIP)":[3],"is":[4,20],"an":[5,57],"autosomal":[6],"dominant":[7],"disorder":[8],"characterized":[9],"by":[10,116,190,243],"insufficient":[11],"porphobilinogen":[12,22],"deaminase":[13],"(PBGD)":[14],"activity.":[15],"When":[16],"hepatic":[17,87],"heme":[18,70,207],"synthesis":[19,71],"induced,":[21],"(PBG)":[23],"and":[24,38,68,75,82,91,100,112,178,187,236],"5-aminolevulinic":[25],"acid":[26],"(ALA)":[27],"accumulate,":[28],"which":[29],"causes":[30],"clinical":[31,253],"symptoms":[32],"such":[33],"as":[34],"abdominal":[35],"pain,":[36],"neuropathy,":[37],"psychiatric":[39],"disturbances.":[40],"Our":[41],"aim":[42],"was":[43,114,145,188],"to":[44,102,138,210,219,228],"investigate":[45],"if":[46],"hepatocyte":[47,122,143,149],"transplantation":[48],"can":[49],"prevent":[50],"or":[51,134],"minimize":[52],"the":[53,83,103,120,141,161,221,231,240],"metabolic":[54,232,241],"alterations":[55],"in":[56,78,128,156,164,168,181,234,255],"AIP":[58,235],"mouse":[59],"model.":[60],"We":[61,124],"transplanted":[62,191],"wild-type":[63],"hepatocytes":[64,162,183,212,226],"into":[65,251],"PBGD-deficient":[66,182,211],"mice":[67],"induced":[69],"with":[72,98],"phenobarbital.":[73],"ALA":[74,111,177],"PBG":[76,113,179],"concentrations":[77],"plasma":[79,169],"were":[80,93,96],"monitored,":[81],"gene":[84],"transcriptions":[85],"of":[86,105,110,131,140,153,160,195,206,224,239,246],"enzymes":[88],"ALAS1,":[89,133],"PBGD,":[90,132],"CYP2A5":[92],"analyzed.":[94],"Results":[95],"compared":[97],"controls":[99],"correlated":[101],"percentage":[104],"engrafted":[106,225],"hepatocytes.":[107],"The":[108,193],"accumulation":[109],"reduced":[115],"approximately":[117],"50%":[118,166],"after":[119],"second":[121],"transplantation.":[123],"detected":[125],"no":[126,203],"difference":[127],"mRNA":[129,199],"levels":[130,200],"CYP2A5.":[135],"Engraftment":[136],"corresponding":[137],"2.7%":[139],"total":[142],"mass":[144],"achieved":[146],"following":[147],"two":[148],"transplantations.":[150],"A":[151],"lack":[152,194],"precursor":[154,170],"production":[155],"less":[157],"than":[158],"3%":[159],"resulted":[163],"a":[165,252],"reduction":[167],"concentrations.":[171],"This":[172],"disproportional":[173],"finding":[174],"suggests":[175,201],"that":[176,202],"produced":[180],"crossed":[184],"cellular":[185],"membranes":[186],"metabolized":[189],"cells.":[192],"effect":[196,254],"on":[197],"enzyme":[198,248],"significant":[204],"efflux":[205],"from":[208],"normal":[209],"takes":[213],"place.":[214],"Further":[215],"studies":[216],"are":[217],"needed":[218,227],"establish":[220],"minimal":[222],"number":[223],"completely":[229],"correct":[230],"abnormality":[233],"whether":[237],"amelioration":[238],"defect":[242],"partial":[244],"restoration":[245],"PBGD":[247],"activity":[249],"translates":[250],"human":[256],"AIP.":[257]},"counts_by_year":[{"year":2025,"cited_by_count":3},{"year":2021,"cited_by_count":3},{"year":2019,"cited_by_count":4},{"year":2017,"cited_by_count":1},{"year":2016,"cited_by_count":5},{"year":2015,"cited_by_count":1},{"year":2014,"cited_by_count":1},{"year":2012,"cited_by_count":1}],"updated_date":"2026-11-04T10:54:05.112534","created_date":"2016-06-24T00:00:00"}
