{"title":"血小板减少症:肌动蛋白动力学缺陷?","authors":"A. Standing","doi":"10.33590/emjhematol/10314114","DOIUrl":null,"url":null,"abstract":"The actin cytoskeleton plays many important roles in the lifecycle of platelets, from biogenesis from megakaryocytes, to activation and clearance from the circulation. It is therefore unsurprising that mutations in genes regulating the dynamics of this cytoskeleton lead to numerous inherited thrombocytopenias. A diverse array of proteins are affected, including actin nucleators, structural proteins, myosin motors, and transcriptional regulators. This review summarises the current understanding of how genetic dysregulation of the actin cytoskeleton can contribute to the pathogenesis of thrombocytopenia.","PeriodicalId":326555,"journal":{"name":"EMJ Hematology","volume":"6 1","pages":"0"},"PeriodicalIF":0.0000,"publicationDate":"2017-08-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"3","resultStr":"{\"title\":\"Thrombocytopenia: A Defect in Actin Dynamics?\",\"authors\":\"A. Standing\",\"doi\":\"10.33590/emjhematol/10314114\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"The actin cytoskeleton plays many important roles in the lifecycle of platelets, from biogenesis from megakaryocytes, to activation and clearance from the circulation. It is therefore unsurprising that mutations in genes regulating the dynamics of this cytoskeleton lead to numerous inherited thrombocytopenias. A diverse array of proteins are affected, including actin nucleators, structural proteins, myosin motors, and transcriptional regulators. This review summarises the current understanding of how genetic dysregulation of the actin cytoskeleton can contribute to the pathogenesis of thrombocytopenia.\",\"PeriodicalId\":326555,\"journal\":{\"name\":\"EMJ Hematology\",\"volume\":\"6 1\",\"pages\":\"0\"},\"PeriodicalIF\":0.0000,\"publicationDate\":\"2017-08-08\",\"publicationTypes\":\"Journal Article\",\"fieldsOfStudy\":null,\"isOpenAccess\":false,\"openAccessPdf\":\"\",\"citationCount\":\"3\",\"resultStr\":null,\"platform\":\"Semanticscholar\",\"paperid\":null,\"PeriodicalName\":\"EMJ Hematology\",\"FirstCategoryId\":\"1085\",\"ListUrlMain\":\"https://doi.org/10.33590/emjhematol/10314114\",\"RegionNum\":0,\"RegionCategory\":null,\"ArticlePicture\":[],\"TitleCN\":null,\"AbstractTextCN\":null,\"PMCID\":null,\"EPubDate\":\"\",\"PubModel\":\"\",\"JCR\":\"\",\"JCRName\":\"\",\"Score\":null,\"Total\":0}","platform":"Semanticscholar","paperid":null,"PeriodicalName":"EMJ Hematology","FirstCategoryId":"1085","ListUrlMain":"https://doi.org/10.33590/emjhematol/10314114","RegionNum":0,"RegionCategory":null,"ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"","JCRName":"","Score":null,"Total":0}
The actin cytoskeleton plays many important roles in the lifecycle of platelets, from biogenesis from megakaryocytes, to activation and clearance from the circulation. It is therefore unsurprising that mutations in genes regulating the dynamics of this cytoskeleton lead to numerous inherited thrombocytopenias. A diverse array of proteins are affected, including actin nucleators, structural proteins, myosin motors, and transcriptional regulators. This review summarises the current understanding of how genetic dysregulation of the actin cytoskeleton can contribute to the pathogenesis of thrombocytopenia.
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