{"id":"https://openalex.org/W2563537467","doi":"https://doi.org/10.1182/blood.v124.21.4200.4200","title":"Aberrant Microtubule Organization and Wiskott-Aldrich Syndrome-like Defects in Platelets and Megakaryocytes of Profilin1-Deficient Mice","display_name":"Aberrant Microtubule Organization and Wiskott-Aldrich Syndrome-like Defects in Platelets and Megakaryocytes of Profilin1-Deficient Mice","publication_year":2014,"publication_date":"2014-12-06","ids":{"openalex":"https://openalex.org/W2563537467","doi":"https://doi.org/10.1182/blood.v124.21.4200.4200","mag":"2563537467"},"language":"en","primary_location":{"id":"doi:10.1182/blood.v124.21.4200.4200","doi":"https://doi.org/10.1182/blood.v124.21.4200.4200","is_oa":false,"landing_page_url":"https://doi.org/10.1182/blood.v124.21.4200.4200","pdf_url":null,"source":{"id":"https://openalex.org/S200071133","display_name":"Blood","issn_l":"0006-4971","issn":["0006-4971","1528-0020"],"is_oa":false,"is_in_doaj":false,"is_core":true,"listed_in":["anvur-scientific","cwts-core","dongbi-a","doyens","jufo-3","ki-jl-3","medline","norway-2","poland-200","russia-white-list-1","vabb-shw"],"host_organization":"https://openalex.org/P4310320990","host_organization_name":"Elsevier BV","host_organization_lineage":["https://openalex.org/P4310320990"],"host_organization_lineage_names":["Elsevier BV"],"type":"journal"},"license":null,"license_id":null,"version":"publishedVersion","is_accepted":true,"is_published":true,"raw_source_name":"Blood","raw_type":"journal-article"},"volume":null,"type":"article","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/A5078018777","display_name":"Simon Stritt","orcid":"https://orcid.org/0000-0002-4299-4934","observed_orcids":["https://orcid.org/0000-0002-4299-4934"]},"institutions":[{"id":"https://openalex.org/I25974101","display_name":"University of W\u00fcrzburg","ror":"https://ror.org/00fbnyb24","country_code":"DE","type":"education","lineage":["https://openalex.org/I25974101"]},{"id":"https://openalex.org/I4210126310","display_name":"Universit\u00e4tsklinikum W\u00fcrzburg","ror":"https://ror.org/03pvr2g57","country_code":"DE","type":"healthcare","lineage":["https://openalex.org/I4210126310"]}],"countries":["DE"],"is_corresponding":false,"raw_author_name":"Simon Stritt","raw_affiliation_strings":["Department of Experimental Biomedicine, University Hospital, University of W\u00fcrzburg, W\u00fcrzburg, Germany"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"Department of Experimental Biomedicine, University Hospital, University of W\u00fcrzburg, W\u00fcrzburg, Germany","institution_ids":["https://openalex.org/I25974101","https://openalex.org/I4210126310"]}]},{"author_position":"middle","author":{"id":"https://openalex.org/A5033473043","display_name":"Markus Bender","orcid":"https://orcid.org/0000-0002-2381-116X","observed_orcids":["https://orcid.org/0000-0002-2381-116X"]},"institutions":[{"id":"https://openalex.org/I1283280774","display_name":"Brigham and Women's Hospital","ror":"https://ror.org/04b6nzv94","country_code":"US","type":"healthcare","lineage":["https://openalex.org/I121934306","https://openalex.org/I1283280774","https://openalex.org/I48633490"]}],"countries":["US"],"is_corresponding":false,"raw_author_name":"Markus Bender","raw_affiliation_strings":["Brigham and Women's Hospital, Boston, MA"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"Brigham and Women's Hospital, Boston, MA","institution_ids":["https://openalex.org/I1283280774"]}]},{"author_position":"middle","author":{"id":"https://openalex.org/A5042115697","display_name":"Paquita Nurden","orcid":"https://orcid.org/0000-0001-5152-1381","observed_orcids":["https://orcid.org/0000-0001-5152-1381"]},"institutions":[{"id":"https://openalex.org/I4210086115","display_name":"Electrophysiology and Heart Modeling Institute","ror":"https://ror.org/00jsv7j98","country_code":"FR","type":"facility","lineage":["https://openalex.org/I1294671590","https://openalex.org/I1326498283","https://openalex.org/I154526488","https://openalex.org/I3018323443","https://openalex.org/I4210086115"]}],"countries":["FR"],"is_corresponding":false,"raw_author_name":"Paquita Nurden","raw_affiliation_strings":["IHU-LIRYC, Pessac, France"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"IHU-LIRYC, Pessac, France","institution_ids":["https://openalex.org/I4210086115"]}]},{"author_position":"middle","author":{"id":"https://openalex.org/A5025484340","display_name":"Judith M.M. van Eeuwijk","orcid":null,"observed_orcids":[]},"institutions":[{"id":"https://openalex.org/I25974101","display_name":"University of W\u00fcrzburg","ror":"https://ror.org/00fbnyb24","country_code":"DE","type":"education","lineage":["https://openalex.org/I25974101"]},{"id":"https://openalex.org/I4210126310","display_name":"Universit\u00e4tsklinikum W\u00fcrzburg","ror":"https://ror.org/03pvr2g57","country_code":"DE","type":"healthcare","lineage":["https://openalex.org/I4210126310"]}],"countries":["DE"],"is_corresponding":false,"raw_author_name":"Judith van Eeuwijk","raw_affiliation_strings":["Department of Experimental Biomedicine, University Hospital, University of W\u00fcrzburg, W\u00fcrzburg, Germany"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"Department of Experimental Biomedicine, University Hospital, University of W\u00fcrzburg, W\u00fcrzburg, Germany","institution_ids":["https://openalex.org/I25974101","https://openalex.org/I4210126310"]}]},{"author_position":"middle","author":{"id":"https://openalex.org/A5017359318","display_name":"Barbara Zieger","orcid":"https://orcid.org/0000-0002-4954-7029","observed_orcids":["https://orcid.org/0000-0002-4954-7029"]},"institutions":[{"id":"https://openalex.org/I2801240073","display_name":"University Medical Center Freiburg","ror":"https://ror.org/03vzbgh69","country_code":"DE","type":"healthcare","lineage":["https://openalex.org/I2801240073"]}],"countries":["DE"],"is_corresponding":false,"raw_author_name":"Barbara Zieger","raw_affiliation_strings":["University Medical Center of Freiburg, Freiburg, Germany"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"University Medical Center of Freiburg, Freiburg, Germany","institution_ids":["https://openalex.org/I2801240073"]}]},{"author_position":"middle","author":{"id":"https://openalex.org/A5089013893","display_name":"Karim Kentouche","orcid":"https://orcid.org/0000-0001-9865-2629","observed_orcids":["https://orcid.org/0000-0001-9865-2629"]},"institutions":[{"id":"https://openalex.org/I4210134900","display_name":"Jena University Hospital","ror":"https://ror.org/035rzkx15","country_code":"DE","type":"healthcare","lineage":["https://openalex.org/I4210134900"]}],"countries":["DE"],"is_corresponding":false,"raw_author_name":"Karim Kentouche","raw_affiliation_strings":["Jena University Hospital, Jena, Germany"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"Jena University Hospital, Jena, Germany","institution_ids":["https://openalex.org/I4210134900"]}]},{"author_position":"middle","author":{"id":"https://openalex.org/A5015284985","display_name":"Harald Schulze","orcid":"https://orcid.org/0000-0003-1285-6407","observed_orcids":["https://orcid.org/0000-0003-1285-6407"]},"institutions":[{"id":"https://openalex.org/I25974101","display_name":"University of W\u00fcrzburg","ror":"https://ror.org/00fbnyb24","country_code":"DE","type":"education","lineage":["https://openalex.org/I25974101"]},{"id":"https://openalex.org/I4210126310","display_name":"Universit\u00e4tsklinikum W\u00fcrzburg","ror":"https://ror.org/03pvr2g57","country_code":"DE","type":"healthcare","lineage":["https://openalex.org/I4210126310"]}],"countries":["DE"],"is_corresponding":false,"raw_author_name":"Harald Schulze","raw_affiliation_strings":["Department of Experimental Biomedicine, University Hospital, University of W\u00fcrzburg, W\u00fcrzburg, Germany"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"Department of Experimental Biomedicine, University Hospital, University of W\u00fcrzburg, W\u00fcrzburg, Germany","institution_ids":["https://openalex.org/I25974101","https://openalex.org/I4210126310"]}]},{"author_position":"middle","author":{"id":"https://openalex.org/A5060043535","display_name":"Henner Morbach","orcid":"https://orcid.org/0000-0002-5819-726X","observed_orcids":["https://orcid.org/0000-0002-5819-726X"]},"institutions":[{"id":"https://openalex.org/I4210126310","display_name":"Universit\u00e4tsklinikum W\u00fcrzburg","ror":"https://ror.org/03pvr2g57","country_code":"DE","type":"healthcare","lineage":["https://openalex.org/I4210126310"]}],"countries":["DE"],"is_corresponding":false,"raw_author_name":"Henner Morbach","raw_affiliation_strings":["Department of Pediatric Haematology, University Hospital W\u00fcrzburg, W\u00fcrzburg, Germany"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"Department of Pediatric Haematology, University Hospital W\u00fcrzburg, W\u00fcrzburg, Germany","institution_ids":["https://openalex.org/I4210126310"]}]},{"author_position":"middle","author":{"id":"https://openalex.org/A5091194864","display_name":"David Stegner","orcid":"https://orcid.org/0000-0003-1059-9865","observed_orcids":["https://orcid.org/0000-0003-1059-9865"]},"institutions":[{"id":"https://openalex.org/I25974101","display_name":"University of W\u00fcrzburg","ror":"https://ror.org/00fbnyb24","country_code":"DE","type":"education","lineage":["https://openalex.org/I25974101"]},{"id":"https://openalex.org/I4210126310","display_name":"Universit\u00e4tsklinikum W\u00fcrzburg","ror":"https://ror.org/03pvr2g57","country_code":"DE","type":"healthcare","lineage":["https://openalex.org/I4210126310"]}],"countries":["DE"],"is_corresponding":false,"raw_author_name":"David Stegner","raw_affiliation_strings":["Department of Experimental Biomedicine, University Hospital, University of W\u00fcrzburg, W\u00fcrzburg, Germany"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"Department of Experimental Biomedicine, University Hospital, University of W\u00fcrzburg, W\u00fcrzburg, Germany","institution_ids":["https://openalex.org/I25974101","https://openalex.org/I4210126310"]}]},{"author_position":"middle","author":{"id":"https://openalex.org/A5032588618","display_name":"Katrin Gertrud Heinze","orcid":"https://orcid.org/0000-0003-2372-6800","observed_orcids":["https://orcid.org/0000-0003-2372-6800"]},"institutions":[{"id":"https://openalex.org/I25974101","display_name":"University of W\u00fcrzburg","ror":"https://ror.org/00fbnyb24","country_code":"DE","type":"education","lineage":["https://openalex.org/I25974101"]}],"countries":["DE"],"is_corresponding":false,"raw_author_name":"Katrin G Heinze","raw_affiliation_strings":["Rudolf Virchow Center for Experimental Biomedicine, University of W\u00fcrzburg, W\u00fcrzburg, Germany"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"Rudolf Virchow Center for Experimental Biomedicine, University of W\u00fcrzburg, W\u00fcrzburg, Germany","institution_ids":["https://openalex.org/I25974101"]}]},{"author_position":"middle","author":{"id":"https://openalex.org/A5030363815","display_name":"Sebastian D\u00fctting","orcid":null,"observed_orcids":[]},"institutions":[{"id":"https://openalex.org/I25974101","display_name":"University of W\u00fcrzburg","ror":"https://ror.org/00fbnyb24","country_code":"DE","type":"education","lineage":["https://openalex.org/I25974101"]},{"id":"https://openalex.org/I4210126310","display_name":"Universit\u00e4tsklinikum W\u00fcrzburg","ror":"https://ror.org/03pvr2g57","country_code":"DE","type":"healthcare","lineage":["https://openalex.org/I4210126310"]}],"countries":["DE"],"is_corresponding":false,"raw_author_name":"Sebastian D\u00fctting","raw_affiliation_strings":["Department of Experimental Biomedicine, University Hospital, University of W\u00fcrzburg, W\u00fcrzburg, Germany"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"Department of Experimental Biomedicine, University Hospital, University of W\u00fcrzburg, W\u00fcrzburg, Germany","institution_ids":["https://openalex.org/I25974101","https://openalex.org/I4210126310"]}]},{"author_position":"middle","author":{"id":"https://openalex.org/A5101764162","display_name":"Shuchi Gupta","orcid":"https://orcid.org/0000-0002-3634-5612","observed_orcids":["https://orcid.org/0000-0002-3634-5612"]},"institutions":[{"id":"https://openalex.org/I25974101","display_name":"University of W\u00fcrzburg","ror":"https://ror.org/00fbnyb24","country_code":"DE","type":"education","lineage":["https://openalex.org/I25974101"]},{"id":"https://openalex.org/I4210126310","display_name":"Universit\u00e4tsklinikum W\u00fcrzburg","ror":"https://ror.org/03pvr2g57","country_code":"DE","type":"healthcare","lineage":["https://openalex.org/I4210126310"]}],"countries":["DE"],"is_corresponding":false,"raw_author_name":"Shuchi Gupta","raw_affiliation_strings":["Department of Experimental Biomedicine, University Hospital, University of W\u00fcrzburg, W\u00fcrzburg, Germany"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"Department of Experimental Biomedicine, University Hospital, University of W\u00fcrzburg, W\u00fcrzburg, Germany","institution_ids":["https://openalex.org/I25974101","https://openalex.org/I4210126310"]}]},{"author_position":"middle","author":{"id":"https://openalex.org/A5089963272","display_name":"Walter Witke","orcid":"https://orcid.org/0000-0001-5018-0060","observed_orcids":["https://orcid.org/0000-0001-5018-0060"]},"institutions":[{"id":"https://openalex.org/I135140700","display_name":"University of Bonn","ror":"https://ror.org/041nas322","country_code":"DE","type":"education","lineage":["https://openalex.org/I135140700"]}],"countries":["DE"],"is_corresponding":false,"raw_author_name":"Walter Witke","raw_affiliation_strings":["University of Bonn, Bonn, Germany"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"University of Bonn, Bonn, Germany","institution_ids":["https://openalex.org/I135140700"]}]},{"author_position":"middle","author":{"id":"https://openalex.org/A5023521073","display_name":"Herv\u00e9 Falet","orcid":"https://orcid.org/0000-0003-0788-9204","observed_orcids":["https://orcid.org/0000-0003-0788-9204"]},"institutions":[{"id":"https://openalex.org/I1283280774","display_name":"Brigham and Women's Hospital","ror":"https://ror.org/04b6nzv94","country_code":"US","type":"healthcare","lineage":["https://openalex.org/I121934306","https://openalex.org/I1283280774","https://openalex.org/I48633490"]},{"id":"https://openalex.org/I136199984","display_name":"Harvard University","ror":"https://ror.org/03vek6s52","country_code":"US","type":"education","lineage":["https://openalex.org/I136199984"]}],"countries":["US"],"is_corresponding":false,"raw_author_name":"Herv\u00e9 Falet","raw_affiliation_strings":["Brigham and Women's Hospital, Boston, MA","Harvard Medical School, Boston, MA"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"Brigham and Women's Hospital, Boston, MA","institution_ids":["https://openalex.org/I1283280774"]},{"raw_affiliation_string":"Harvard Medical School, Boston, MA","institution_ids":["https://openalex.org/I136199984"]}]},{"author_position":"middle","author":{"id":"https://openalex.org/A5029004287","display_name":"Alain Fischer","orcid":"https://orcid.org/0000-0003-4025-5983","observed_orcids":["https://orcid.org/0000-0003-4025-5983"]},"institutions":[{"id":"https://openalex.org/I1288880153","display_name":"H\u00f4pital Necker-Enfants Malades","ror":"https://ror.org/05tr67282","country_code":"FR","type":"healthcare","lineage":["https://openalex.org/I1288880153","https://openalex.org/I4210097159","https://openalex.org/I4210120235"]}],"countries":["FR"],"is_corresponding":false,"raw_author_name":"Alain Fischer","raw_affiliation_strings":["Unit\u00e9 d'Immunologie et d'H\u00e9matologie P\u00e9diatrique, H\u00f4pital Universitaire Necker-Enfants Malades, Paris, France"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"Unit\u00e9 d'Immunologie et d'H\u00e9matologie P\u00e9diatrique, H\u00f4pital Universitaire Necker-Enfants Malades, Paris, France","institution_ids":["https://openalex.org/I1288880153"]}]},{"author_position":"middle","author":{"id":"https://openalex.org/A5071191923","display_name":"John H. Hartwig","orcid":null,"observed_orcids":[]},"institutions":[{"id":"https://openalex.org/I1283280774","display_name":"Brigham and Women's Hospital","ror":"https://ror.org/04b6nzv94","country_code":"US","type":"healthcare","lineage":["https://openalex.org/I121934306","https://openalex.org/I1283280774","https://openalex.org/I48633490"]}],"countries":["US"],"is_corresponding":false,"raw_author_name":"John H. Hartwig","raw_affiliation_strings":["Brigham & Women's Hospital, Boston, MA"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"Brigham & Women's Hospital, Boston, MA","institution_ids":["https://openalex.org/I1283280774"]}]},{"author_position":"last","author":{"id":"https://openalex.org/A5008888080","display_name":"Bernhard Nieswandt","orcid":"https://orcid.org/0000-0003-1454-7413","observed_orcids":["https://orcid.org/0000-0003-1454-7413"]},"institutions":[{"id":"https://openalex.org/I25974101","display_name":"University of W\u00fcrzburg","ror":"https://ror.org/00fbnyb24","country_code":"DE","type":"education","lineage":["https://openalex.org/I25974101"]},{"id":"https://openalex.org/I4210126310","display_name":"Universit\u00e4tsklinikum W\u00fcrzburg","ror":"https://ror.org/03pvr2g57","country_code":"DE","type":"healthcare","lineage":["https://openalex.org/I4210126310"]}],"countries":["DE"],"is_corresponding":false,"raw_author_name":"Bernhard Nieswandt","raw_affiliation_strings":["Department of Experimental Biomedicine, University Hospital, University of W\u00fcrzburg, W\u00fcrzburg, Germany"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"Department of Experimental Biomedicine, University Hospital, University of W\u00fcrzburg, W\u00fcrzburg, Germany","institution_ids":["https://openalex.org/I25974101","https://openalex.org/I4210126310"]}]}],"institutions":[],"countries_distinct_count":3,"institutions_distinct_count":9,"corresponding_author_ids":[],"corresponding_institution_ids":[],"apc_list":{"value":5500,"currency":"USD","value_usd":5500},"apc_paid":null,"fwci":0.0,"has_fulltext":false,"cited_by_count":0,"citation_normalized_percentile":{"value":0.33463288,"is_in_top_1_percent":false,"is_in_top_10_percent":false},"cited_by_percentile_year":null,"biblio":{"volume":"124","issue":"21","first_page":"4200","last_page":"4200"},"is_retracted":false,"is_paratext":false,"is_xpac":false,"primary_topic":{"id":"https://openalex.org/T10746","display_name":"Platelet Disorders and Treatments","score":1.0,"subfield":{"id":"https://openalex.org/subfields/2720","display_name":"Hematology"},"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/T10746","display_name":"Platelet Disorders and Treatments","score":1.0,"subfield":{"id":"https://openalex.org/subfields/2720","display_name":"Hematology"},"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/T11725","display_name":"Immunodeficiency and Autoimmune Disorders","score":9.999999747378752e-05,"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/T10881","display_name":"Blood Coagulation and Thrombosis Mechanisms","score":0.0,"subfield":{"id":"https://openalex.org/subfields/2720","display_name":"Hematology"},"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/wiskott-aldrich-syndrome","display_name":"Wiskott-Aldrich syndrome","score":0.9200000166893005},{"id":"https://openalex.org/keywords/profilin-1","display_name":"profilin-1","score":0.9409999847412109},{"id":"https://openalex.org/keywords/microtubule-organization","display_name":"microtubule organization","score":0.7960000038146973},{"id":"https://openalex.org/keywords/microtubule-stability","display_name":"microtubule stability","score":0.953000009059906},{"id":"https://openalex.org/keywords/platelet-size","display_name":"platelet size","score":0.7260000109672546},{"id":"https://openalex.org/keywords/platelet-clearance","display_name":"platelet clearance","score":0.6790000200271606},{"id":"https://openalex.org/keywords/megakaryocytes","display_name":"megakaryocytes","score":0.8859999775886536},{"id":"https://openalex.org/keywords/wasps","display_name":"wasps","score":0.6449999809265137},{"id":"https://openalex.org/keywords/actin-regulation","display_name":"actin regulation","score":0.6880000233650208},{"id":"https://openalex.org/keywords/platelet-formation","display_name":"platelet formation","score":0.6320000290870667},{"id":"https://openalex.org/keywords/trichostatin-a","display_name":"trichostatin A","score":0.7950000166893005},{"id":"https://openalex.org/keywords/paclitaxel","display_name":"paclitaxel","score":0.8690000176429749}],"concepts":[{"id":"https://openalex.org/C89560881","wikidata":"https://www.wikidata.org/wiki/Q101026","display_name":"Platelet","level":2,"score":0.803364634513855},{"id":"https://openalex.org/C2780939953","wikidata":"https://www.wikidata.org/wiki/Q8027658","display_name":"Wiskott\u2013Aldrich syndrome protein","level":5,"score":0.7480166554450989},{"id":"https://openalex.org/C20418707","wikidata":"https://www.wikidata.org/wiki/Q189933","display_name":"Microtubule","level":2,"score":0.6271551847457886},{"id":"https://openalex.org/C2777865847","wikidata":"https://www.wikidata.org/wiki/Q425894","display_name":"Trichostatin A","level":5,"score":0.6130949258804321},{"id":"https://openalex.org/C2778153459","wikidata":"https://www.wikidata.org/wiki/Q953638","display_name":"Wiskott\u2013Aldrich syndrome","level":3,"score":0.5348339080810547},{"id":"https://openalex.org/C95444343","wikidata":"https://www.wikidata.org/wiki/Q7141","display_name":"Cell biology","level":1,"score":0.4762406647205353},{"id":"https://openalex.org/C203014093","wikidata":"https://www.wikidata.org/wiki/Q101929","display_name":"Immunology","level":1,"score":0.4543239176273346},{"id":"https://openalex.org/C86803240","wikidata":"https://www.wikidata.org/wiki/Q420","display_name":"Biology","level":0,"score":0.4473692774772644},{"id":"https://openalex.org/C153911025","wikidata":"https://www.wikidata.org/wiki/Q7202","display_name":"Molecular biology","level":1,"score":0.4452369511127472},{"id":"https://openalex.org/C2778305200","wikidata":"https://www.wikidata.org/wiki/Q174378","display_name":"Histone deacetylase","level":4,"score":0.43869689106941223},{"id":"https://openalex.org/C185592680","wikidata":"https://www.wikidata.org/wiki/Q2329","display_name":"Chemistry","level":0,"score":0.3350200057029724},{"id":"https://openalex.org/C64927066","wikidata":"https://www.wikidata.org/wiki/Q36293","display_name":"Histone","level":3,"score":0.2616865932941437},{"id":"https://openalex.org/C104317684","wikidata":"https://www.wikidata.org/wiki/Q7187","display_name":"Gene","level":2,"score":0.2091343104839325},{"id":"https://openalex.org/C1491633281","wikidata":"https://www.wikidata.org/wiki/Q7868","display_name":"Cell","level":2,"score":0.19095313549041748},{"id":"https://openalex.org/C54355233","wikidata":"https://www.wikidata.org/wiki/Q7162","display_name":"Genetics","level":1,"score":0.16299307346343994},{"id":"https://openalex.org/C142669718","wikidata":"https://www.wikidata.org/wiki/Q154626","display_name":"Cytoskeleton","level":3,"score":0.1470567286014557},{"id":"https://openalex.org/C2993400109","wikidata":"https://www.wikidata.org/wiki/Q329638","display_name":"Actin cytoskeleton","level":4,"score":0.10803118348121643}],"mesh":[],"locations_count":1,"locations":[{"id":"doi:10.1182/blood.v124.21.4200.4200","doi":"https://doi.org/10.1182/blood.v124.21.4200.4200","is_oa":false,"landing_page_url":"https://doi.org/10.1182/blood.v124.21.4200.4200","pdf_url":null,"source":{"id":"https://openalex.org/S200071133","display_name":"Blood","issn_l":"0006-4971","issn":["0006-4971","1528-0020"],"is_oa":false,"is_in_doaj":false,"is_core":true,"listed_in":["anvur-scientific","cwts-core","dongbi-a","doyens","jufo-3","ki-jl-3","medline","norway-2","poland-200","russia-white-list-1","vabb-shw"],"host_organization":"https://openalex.org/P4310320990","host_organization_name":"Elsevier BV","host_organization_lineage":["https://openalex.org/P4310320990"],"host_organization_lineage_names":["Elsevier BV"],"type":"journal"},"license":null,"license_id":null,"version":"publishedVersion","is_accepted":true,"is_published":true,"raw_source_name":"Blood","raw_type":"journal-article"}],"best_oa_location":null,"sustainable_development_goals":[{"id":"https://metadata.un.org/sdg/3","display_name":"Good health and well-being","score":0.9391000270843506}],"sustainable_development_goals_aurora":[],"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/W2040989248","https://openalex.org/W74607343","https://openalex.org/W3177107356","https://openalex.org/W1969172568","https://openalex.org/W2103771488","https://openalex.org/W7097689669","https://openalex.org/W7113071626","https://openalex.org/W2753909456","https://openalex.org/W2590589078","https://openalex.org/W2083197627"],"abstract_inverted_index":{"Abstract":[0],"Wiskott-Aldrich":[1],"syndrome":[2],"(WAS)":[3],"is":[4,11,58],"a":[5,59,185,264,272],"rare,":[6],"X-chromosomal":[7],"recessive":[8],"disorder":[9],"which":[10],"caused":[12],"by":[13,21,112],"mutations":[14,48],"in":[15,132,184,203,208,248,281],"the":[16,43,74,119,124,128,159,175,199,241,277],"WAS":[17,34,47,75,98,141,209,252,282],"gene":[18],"and":[19,24,27,41,49,69,114,140,146,223,232,269],"characterized":[20],"eczema,":[22],"immunodeficiency":[23],"microthrombocytopenia":[25,53],"(Thrasher":[26],"Burns,":[28],"Nat":[29],"Rev":[30],"Immunol":[31],"2010).":[32],"Interestingly,":[33,95],"protein":[35,62,76,78],"(WASp)-deficient":[36],"mice":[37,100,139,205],"have":[38],"normal-sized":[39],"platelets":[40,136,168,249],"thus":[42],"molecular":[44],"link":[45],"between":[46],"its":[50],"central":[51],"hallmark":[52],"remains":[54],"elusive.":[55],"Profilin1":[56],"(Pfn1)":[57],"key":[60],"actin-regulating":[61],"that,":[63],"besides":[64],"actin,":[65],"interacts":[66],"with":[67,101,169],"phosphoinositides":[68],"multiple":[70],"proline-rich":[71],"proteins":[72],"including":[73],"(WASp)/WASp-interacting":[77],"(WIP)":[79],"complex":[80],"(Witke,":[81],"Trends":[82],"Cell":[83],"Biol":[84],"2004;":[85],"Ramesh":[86],"et":[87],"al.,":[88],"Proc":[89],"Natl":[90],"Acad":[91],"Sci":[92],"USA,":[93],"1997).":[94],"similar":[96],"to":[97,229,271,290],"patients,":[99],"Pfn1-null":[102,138],"megakaryocytes/platelets":[103],"suffered":[104],"from":[105,137],"microthrombocytopenia.":[106],"We":[107,149],"identified":[108],"accelerated":[109],"platelet":[110,116,130,187,201,278],"clearance":[111],"macrophages":[113],"pre-mature":[115],"release":[117],"into":[118],"bone":[120],"marrow":[121],"compartment":[122],"as":[123,174,263],"major":[125],"cause":[126],"of":[127,162,166,225,235,239,244,250,261,266,288],"reduced":[129,160,200],"count":[131],"Pfn1-deficient":[133,163,204],"mice.":[134],"Both,":[135],"patients":[142],"contained":[143],"abnormally":[144],"organized":[145],"hyperstable":[147],"microtubules.":[148,236],"next":[150],"tested,":[151],"if":[152],"increased":[153,193,230],"microtubule":[154,170,194,267],"stability":[155,195,231],"could":[156,196],"account":[157,197],"for":[158,198],"size":[161,202],"platelets.":[164],"Treatment":[165],"control":[167],"stabilizing":[171],"toxins,":[172],"such":[173],"histone-deacetylase":[176],"inhibitor":[177],"trichostatin":[178],"A":[179],"(TSA)":[180],"or":[181],"taxol":[182],"resulted":[183],"decreased":[186],"size.":[188],"This":[189],"finding":[190],"indicates":[191],"that":[192,217],"but":[206],"also":[207],"patients.":[210,253,283],"Based":[211],"on":[212],"these":[213,255],"results":[214,256],"we":[215],"speculate":[216],"WASp":[218],"might":[219],"modulate":[220],"Pfn1":[221,245,262],"function":[222,260],"dysregulation":[224],"this":[226],"interaction":[227],"leads":[228],"altered":[233,247],"organization":[234,268],"In":[237],"support":[238],"this,":[240],"subcellular":[242],"localization":[243],"was":[246],"three":[251],"Together,":[254],"reveal":[257],"an":[258],"unexpected":[259],"regulator":[265],"point":[270],"previously":[273],"unrecognized":[274],"mechanism":[275],"underlying":[276],"formation":[279],"defect":[280],"Disclosures":[284],"No":[285],"relevant":[286],"conflicts":[287],"interest":[289],"declare.":[291]},"counts_by_year":[],"updated_date":"2026-10-08T09:06:37.294493","created_date":"2017-01-06T00:00:00"}
