{"id":"https://openalex.org/W2403672035","doi":null,"title":"The catabolism of C1(-)-inhibitor and the pathogenesis of hereditary angio-edema.","display_name":"The catabolism of C1(-)-inhibitor and the pathogenesis of hereditary angio-edema.","publication_year":1984,"publication_date":"1984-01-01","ids":{"openalex":"https://openalex.org/W2403672035","mag":"2403672035","pmid":"https://pubmed.ncbi.nlm.nih.gov/6587741"},"language":"en","primary_location":{"id":"pmid:6587741","doi":null,"is_oa":false,"landing_page_url":"https://pubmed.ncbi.nlm.nih.gov/6587741","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":"Acta pathologica, microbiologica, et immunologica Scandinavica. Supplement","raw_type":"Comparative Study"},"volume":null,"type":"article","indexed_in":["pubmed"],"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/A5076662711","display_name":"P J Lachmann","orcid":null,"observed_orcids":[]},"institutions":[],"countries":[],"is_corresponding":false,"raw_author_name":"P J Lachmann","raw_affiliation_strings":[],"raw_orcid":null,"affiliations":[]},{"author_position":"last","author":{"id":"https://openalex.org/A5111433586","display_name":"Fred S. Rosen","orcid":null,"observed_orcids":[]},"institutions":[],"countries":[],"is_corresponding":false,"raw_author_name":"F S Rosen","raw_affiliation_strings":[],"raw_orcid":null,"affiliations":[]}],"institutions":[],"countries_distinct_count":0,"institutions_distinct_count":0,"corresponding_author_ids":[],"corresponding_institution_ids":[],"apc_list":null,"apc_paid":null,"fwci":0.6551,"has_fulltext":false,"cited_by_count":31,"citation_normalized_percentile":{"value":0.72185154,"is_in_top_1_percent":false,"is_in_top_10_percent":false},"cited_by_percentile_year":{"min":89,"max":96},"biblio":{"volume":"284","issue":null,"first_page":"35","last_page":"9"},"is_retracted":false,"is_paratext":false,"is_xpac":false,"primary_topic":{"id":"https://openalex.org/T11917","display_name":"Coagulation, Bradykinin, Polyphosphates, and Angioedema","score":1.0,"subfield":{"id":"https://openalex.org/subfields/2716","display_name":"Genetics"},"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/T11917","display_name":"Coagulation, Bradykinin, Polyphosphates, and Angioedema","score":1.0,"subfield":{"id":"https://openalex.org/subfields/2716","display_name":"Genetics"},"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/T10993","display_name":"Complement system in diseases","score":0.0,"subfield":{"id":"https://openalex.org/subfields/2403","display_name":"Immunology"},"field":{"id":"https://openalex.org/fields/24","display_name":"Immunology and Microbiology"},"domain":{"id":"https://openalex.org/domains/1","display_name":"Life Sciences"}},{"id":"https://openalex.org/T10620","display_name":"Protease and Inhibitor Mechanisms","score":0.0,"subfield":{"id":"https://openalex.org/subfields/1306","display_name":"Cancer Research"},"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/c1-inhibitor","display_name":"C1 inhibitor","score":0.8999999761581421},{"id":"https://openalex.org/keywords/hereditary-angioedema","display_name":"hereditary angioedema","score":0.9929999709129333},{"id":"https://openalex.org/keywords/protein-degradation","display_name":"protein degradation","score":0.8100000023841858},{"id":"https://openalex.org/keywords/enzyme-inhibitor-complex","display_name":"enzyme-inhibitor complex","score":0.8009999990463257},{"id":"https://openalex.org/keywords/hemizygotes","display_name":"hemizygotes","score":0.9440000057220459},{"id":"https://openalex.org/keywords/protein-turnover","display_name":"protein turnover","score":0.7419999837875366},{"id":"https://openalex.org/keywords/enzyme-activation","display_name":"enzyme activation","score":0.6940000057220459},{"id":"https://openalex.org/keywords/pathogenesis","display_name":"pathogenesis","score":0.6000000238418579}],"concepts":[{"id":"https://openalex.org/C96942376","wikidata":"https://www.wikidata.org/wiki/Q21130714","display_name":"Catabolism","level":3,"score":0.8880637288093567},{"id":"https://openalex.org/C27502469","wikidata":"https://www.wikidata.org/wiki/Q906343","display_name":"Dysfunctional family","level":2,"score":0.7472856044769287},{"id":"https://openalex.org/C2780942790","wikidata":"https://www.wikidata.org/wiki/Q372016","display_name":"Pathogenesis","level":2,"score":0.6169673800468445},{"id":"https://openalex.org/C181199279","wikidata":"https://www.wikidata.org/wiki/Q8047","display_name":"Enzyme","level":2,"score":0.567571759223938},{"id":"https://openalex.org/C185592680","wikidata":"https://www.wikidata.org/wiki/Q2329","display_name":"Chemistry","level":0,"score":0.40478867292404175},{"id":"https://openalex.org/C55493867","wikidata":"https://www.wikidata.org/wiki/Q7094","display_name":"Biochemistry","level":1,"score":0.39164087176322937},{"id":"https://openalex.org/C86803240","wikidata":"https://www.wikidata.org/wiki/Q420","display_name":"Biology","level":0,"score":0.34898459911346436},{"id":"https://openalex.org/C126322002","wikidata":"https://www.wikidata.org/wiki/Q11180","display_name":"Internal medicine","level":1,"score":0.3146725296974182},{"id":"https://openalex.org/C71924100","wikidata":"https://www.wikidata.org/wiki/Q11190","display_name":"Medicine","level":0,"score":0.2572707533836365},{"id":"https://openalex.org/C70410870","wikidata":"https://www.wikidata.org/wiki/Q199906","display_name":"Clinical psychology","level":1,"score":0.0}],"mesh":[{"descriptor_ui":"D000799","descriptor_name":"Angioedema","qualifier_ui":"Q000209","qualifier_name":"etiology","is_major_topic":true},{"descriptor_ui":"D000799","descriptor_name":"Angioedema","qualifier_ui":"Q000235","qualifier_name":"genetics","is_major_topic":false},{"descriptor_ui":"D000799","descriptor_name":"Angioedema","qualifier_ui":"Q000276","qualifier_name":"immunology","is_major_topic":false},{"descriptor_ui":"D003167","descriptor_name":"Complement Activation","qualifier_ui":null,"qualifier_name":null,"is_major_topic":false},{"descriptor_ui":"D003174","descriptor_name":"Complement C1 Inactivator Proteins","qualifier_ui":"Q000235","qualifier_name":"genetics","is_major_topic":false},{"descriptor_ui":"D003174","descriptor_name":"Complement C1 Inactivator Proteins","qualifier_ui":"Q000378","qualifier_name":"metabolism","is_major_topic":true},{"descriptor_ui":"D006579","descriptor_name":"Heterozygote","qualifier_ui":null,"qualifier_name":null,"is_major_topic":false},{"descriptor_ui":"D006801","descriptor_name":"Humans","qualifier_ui":null,"qualifier_name":null,"is_major_topic":false},{"descriptor_ui":"D008954","descriptor_name":"Models, Biological","qualifier_ui":null,"qualifier_name":null,"is_major_topic":false}],"locations_count":2,"locations":[{"id":"pmid:6587741","doi":null,"is_oa":false,"landing_page_url":"https://pubmed.ncbi.nlm.nih.gov/6587741","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":"Acta pathologica, microbiologica, et immunologica Scandinavica. Supplement","raw_type":"Comparative Study"},{"id":"mag:2403672035","doi":null,"is_oa":false,"landing_page_url":"https://pubmed.ncbi.nlm.nih.gov/6587741","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":null,"is_accepted":false,"is_published":false,"raw_source_name":"PubMed","raw_type":null}],"best_oa_location":null,"sustainable_development_goals":[{"id":"https://metadata.un.org/sdg/3","score":0.9391000270843506,"display_name":"Good health and well-being"}],"study_designs":[],"awards":[],"funders":[],"has_content":{"grobid_xml":false,"pdf":false},"content_urls":null,"referenced_works_count":0,"referenced_works":[],"related_works":["https://openalex.org/W1999681026","https://openalex.org/W4300457475","https://openalex.org/W2065956940","https://openalex.org/W2018174393","https://openalex.org/W2151388112","https://openalex.org/W2439506926","https://openalex.org/W2164980913","https://openalex.org/W2061006221","https://openalex.org/W2181784814","https://openalex.org/W247198690"],"abstract_inverted_index":{"A":[0],"sufficient":[1],"explanation":[2],"for":[3,81,144],"the":[4,15,20,26,42,48,57,61,67,93,112,117,121,124,128,131,140,145,151,176],"observations":[5],"that":[6,14,37],"HAE":[7],"is":[8,193],"a":[9,38,52,173],"dominantly":[10],"transmitted":[11],"disease":[12,125],"and":[13,80,88,108,111,168,171],"hemizygotes":[16],"have":[17],"levels":[18],"of":[19,23,28,30,41,44,51,56,66,73,77,92,95,123,139,150,166,175,178,180,183,187],"normal":[21,31,107,169],"protein":[22,130,133,142],"only":[24],"in":[25,86,89,161,190],"region":[27],"15%-20%":[29],"can":[32,100,158],"be":[33,70,101,159],"given":[34],"by":[35,163],"proposing":[36],"substantial":[39],"proportion":[40],"catabolism":[43,68,156],"C1(-)-esterase":[45],"inhibitor":[46,62,74,153,170],"involves":[47],"prior":[49],"formation":[50],"complex":[53,154],"with":[54,59,106,116,127],"one":[55],"enzymes":[58],"which":[60],"reacts.":[63],"This":[64],"part":[65],"will":[69],"largely":[71],"independent":[72],"concentration,":[75],"i.e.":[76],"zero":[78,97],"order,":[79],"this":[82,96,181,188],"reason":[83],"occurs":[84],"similarly":[85],"normals":[87],"hemizygotes.":[90],"Estimates":[91],"extent":[94,149,177],"order":[98],"metabolism":[99],"obtained":[102],"from":[103],"turnover":[104],"data":[105],"dysfunctional":[109,132,167],"C1(-)-inhibitor":[110],"results":[113],"are":[114],"consistent":[115],"observed":[118],"levels.":[119],"In":[120],"form":[122],"associated":[126],"dysfunction":[129],"makes":[134],"up":[135],"more":[136],"than":[137],"85%":[138],"total":[141],"found":[143],"same":[146],"reason.":[147],"The":[148,185],"enzyme":[152],"dependent":[155],"(RO)":[157],"determined":[160],"vivo":[162],"simultaneous":[164],"turnovers":[165],"gives":[172],"measure":[174],"activation":[179],"group":[182],"enzymes.":[184],"value":[186],"technique":[189],"clinical":[191],"practice":[192],"described":[194],"elsewhere.":[195]},"counts_by_year":[{"year":2025,"cited_by_count":1},{"year":2023,"cited_by_count":1},{"year":2019,"cited_by_count":2},{"year":2016,"cited_by_count":2},{"year":2014,"cited_by_count":1}],"updated_date":"2026-10-31T12:09:11.096373","created_date":"2016-06-24T00:00:00"}