{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,29]],"date-time":"2026-08-29T14:02:37Z","timestamp":1788012157164,"version":"build-2784847793"},"reference-count":25,"publisher":"Springer Science and Business Media LLC","issue":"2","license":[{"start":{"date-parts":[[2024,1,4]],"date-time":"2024-01-04T00:00:00Z","timestamp":1704326400000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.springernature.com\/gp\/researchers\/text-and-data-mining"},{"start":{"date-parts":[[2024,1,4]],"date-time":"2024-01-04T00:00:00Z","timestamp":1704326400000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/www.springernature.com\/gp\/researchers\/text-and-data-mining"}],"content-domain":{"domain":["link.springer.com"],"crossmark-restriction":false},"short-container-title":["Ann Hematol"],"published-print":{"date-parts":[[2024,2]]},"DOI":"10.1007\/s00277-023-05607-6","type":"journal-article","created":{"date-parts":[[2024,1,4]],"date-time":"2024-01-04T10:02:37Z","timestamp":1704362557000},"page":"653-662","update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":3,"title":["Analysis of PROS1 mutations and clinical characteristics in three Chinese families with hereditary protein S deficiency"],"prefix":"10.1007","volume":"103","author":[{"given":"Fei","family":"Xu","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Xingxing","family":"Zhou","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yanhui","family":"Jin","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lihong","family":"Yang","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jingye","family":"Pan","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mingshan","family":"Wang","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Xiaoli","family":"Chen","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2024,1,4]]},"reference":[{"issue":"2","key":"5607_CR1","doi-asserted-by":"publisher","first-page":"222","DOI":"10.1159\/000508525","volume":"144","author":"D Juhl","year":"2021","unstructured":"Juhl D, Kuta P, Shneyder M et al (2021) Two novel variants in the protein S gene PROS1 are associated with protein S deficiency and thrombophilia. Acta Haematol 144(2):222\u2013226. https:\/\/doi.org\/10.1159\/000508525","journal-title":"Acta Haematol"},{"issue":"2018","key":"5607_CR2","doi-asserted-by":"publisher","first-page":"5217301","DOI":"10.1155\/2018\/5217301","volume":"23","author":"K Ameku","year":"2018","unstructured":"Ameku K, Higa M (2018) Rivaroxaban treatment for warfarin-refractory thrombosis in a patient with hereditary protein S deficiency. Case Rep Hematol 23(2018):5217301. https:\/\/doi.org\/10.1155\/2018\/5217301","journal-title":"Case Rep Hematol"},{"issue":"Suppl 1","key":"5607_CR3","doi-asserted-by":"publisher","first-page":"25","DOI":"10.1186\/s12959-016-0109-x","volume":"14","author":"T Miyata","year":"2016","unstructured":"Miyata T, Maruyama K, Banno F et al (2016) Thrombophilia in East Asian countries: are there any genetic differences in these countries? Thromb J 14(Suppl 1):25. https:\/\/doi.org\/10.1186\/s12959-016-0109-x","journal-title":"Thromb J"},{"issue":"5","key":"5607_CR4","doi-asserted-by":"publisher","first-page":"339","DOI":"10.1097\/MOH.0000000000000663","volume":"28","author":"R Majumder","year":"2021","unstructured":"Majumder R, Nguyen T (2021) Protein S: function, regulation, and clinical perspectives. Curr Opin Hematol 28(5):339\u2013344. https:\/\/doi.org\/10.1097\/MOH.0000000000000663","journal-title":"Curr Opin Hematol"},{"issue":"3","key":"5607_CR5","doi-asserted-by":"publisher","first-page":"e7472","DOI":"10.7759\/cureus.7472","volume":"12","author":"Z Majid","year":"2020","unstructured":"Majid Z, Tahir F, Ahmed J et al (2020) Protein C deficiency as a risk factor for stroke in young adults: a review. Cureus 12(3):e7472. https:\/\/doi.org\/10.7759\/cureus.7472","journal-title":"Cureus"},{"issue":"4","key":"5607_CR6","first-page":"459","volume":"22","author":"E Wypasek","year":"2013","unstructured":"Wypasek E, Undas A (2013) Protein C and protein S deficiency - practical diagnostic issues. Adv Clin Exp Med 22(4):459\u2013467","journal-title":"Adv Clin Exp Med"},{"issue":"11","key":"5607_CR7","doi-asserted-by":"publisher","first-page":"2801","DOI":"10.1111\/jth.15025","volume":"18","author":"M Gierula","year":"2020","unstructured":"Gierula M, Ahnstr\u00f6m J (2020) Anticoagulant protein S-new insights on interactions and functions. J Thromb Haemost 18(11):2801\u20132811. https:\/\/doi.org\/10.1111\/jth.15025","journal-title":"J Thromb Haemost"},{"issue":"3","key":"5607_CR8","doi-asserted-by":"publisher","first-page":"409","DOI":"10.1111\/j.1365-2141.2008.07366.x","volume":"143","author":"M Takeyama","year":"2008","unstructured":"Takeyama M, Nogami K, Saenko EL et al (2008) Protein S down-regulates factor Xase activity independent of activated protein C: specific binding of factor VIII(a) to protein S inhibits interactions with factor IXa. Br J Haematol 143(3):409\u2013420. https:\/\/doi.org\/10.1111\/j.1365-2141.2008.07366.x","journal-title":"Br J Haematol"},{"issue":"10294","key":"5607_CR9","doi-asserted-by":"publisher","first-page":"64","DOI":"10.1016\/S0140-6736(20)32658-1","volume":"398","author":"F Khan","year":"2021","unstructured":"Khan F, Tritschler T, Kahn SR et al (2021) Venous thromboembolism. Lancet 398(10294):64\u201377. https:\/\/doi.org\/10.1016\/S0140-6736(20)32658-1","journal-title":"Lancet"},{"issue":"10","key":"5607_CR10","doi-asserted-by":"publisher","first-page":"1181","DOI":"10.1042\/CS20200403","volume":"134","author":"S Labrouche-Colomer","year":"2020","unstructured":"Labrouche-Colomer S, Soukarieh O, Proust C et al (2020) A novel rare c.-39C>T mutation in the PROS1 5\u2032UTR causing PS deficiency by creating a new upstream translation initiation codon. Clin Sci (Lond) 134(10):1181\u20131190. https:\/\/doi.org\/10.1042\/CS20200403","journal-title":"Clin Sci (Lond)"},{"issue":"1","key":"5607_CR11","doi-asserted-by":"publisher","first-page":"52","DOI":"10.1186\/s12959-023-00494-3","volume":"21","author":"L Li","year":"2023","unstructured":"Li L, Wu X, Wu W et al (2023) A case-report of the unprovoked thrombotic event in a patient with thymoma and severe FVII deficiency. Thromb J 21(1):52. https:\/\/doi.org\/10.1186\/s12959-023-00494-3","journal-title":"Thromb J"},{"issue":"1","key":"5607_CR12","doi-asserted-by":"publisher","first-page":"7","DOI":"10.1136\/jclinpath-2019-205956","volume":"73","author":"J Zhou","year":"2020","unstructured":"Zhou J, Shen W, Gu Y et al (2020) Compound heterozygous mutations identified in severe type I protein S deficiency impaired the secretion of protein S. J Clin Pathol 73(1):7\u201313. https:\/\/doi.org\/10.1136\/jclinpath-2019-205956","journal-title":"J Clin Pathol"},{"issue":"3","key":"5607_CR13","doi-asserted-by":"publisher","first-page":"272","DOI":"10.1007\/s12185-016-2111-2","volume":"105","author":"M Ikejiri","year":"2017","unstructured":"Ikejiri M, Wada H, Yamada N et al (2017) High prevalence of congenital thrombophilia in patients with pregnancy-related or idiopathic venous thromboembolism\/pulmonary embolism. Int J Hematol 105(3):272\u2013279. https:\/\/doi.org\/10.1007\/s12185-016-2111-2","journal-title":"Int J Hematol"},{"issue":"4","key":"5607_CR14","doi-asserted-by":"publisher","first-page":"750","DOI":"10.1111\/j.1538-7836.2010.03743.x","volume":"8","author":"LF Maurissen","year":"2010","unstructured":"Maurissen LF, Castoldi E, Simioni P et al (2010) Thrombin generation-based assays to measure the activity of the TFPI-protein S pathway in plasma from normal and protein S-deficient individuals. J Thromb Haemost 8(4):750\u2013758. https:\/\/doi.org\/10.1111\/j.1538-7836.2010.03743.x","journal-title":"J Thromb Haemost"},{"issue":"6-7","key":"5607_CR15","doi-asserted-by":"publisher","first-page":"612","DOI":"10.1016\/j.bbagrm.2013.02.005","volume":"1829","author":"C Schweingruber","year":"2013","unstructured":"Schweingruber C, Rufener SC, Z\u00fcnd D et al (2013) Nonsense-mediated mRNA decay - mechanisms of substrate mRNA recognition and degradation in mammalian cells. Biochim Biophys Acta 1829(6-7):612\u2013623. https:\/\/doi.org\/10.1016\/j.bbagrm.2013.02.005","journal-title":"Biochim Biophys Acta"},{"issue":"Pt 3","key":"5607_CR16","doi-asserted-by":"publisher","first-page":"757","DOI":"10.1042\/bj3490757","volume":"349","author":"TM Hackeng","year":"2000","unstructured":"Hackeng TM, Yegneswaran S, Johnson AE et al (2000) Conformational changes in activated protein C caused by binding of the first epidermal growth factor-like module of protein S. Biochem J 349(Pt 3):757\u2013764. https:\/\/doi.org\/10.1042\/bj3490757","journal-title":"Biochem J"},{"issue":"2","key":"5607_CR17","doi-asserted-by":"publisher","first-page":"590","DOI":"10.1055\/s-0038-1649780","volume":"74","author":"T Yamazaki","year":"1995","unstructured":"Yamazaki T, Hamaguchi M, Katsumi A et al (1995) A quantitative protein S deficiency associated with a novel nonsense mutation and markedly reduced levels of mutated mRNA. Thromb Haemost 74(2):590\u2013595","journal-title":"Thromb Haemost"},{"issue":"3","key":"5607_CR18","doi-asserted-by":"publisher","first-page":"259","DOI":"10.1002\/humu.20136","volume":"25","author":"E Biguzzi","year":"2005","unstructured":"Biguzzi E, Razzari C, Lane DA et al (2005) Molecular diversity and thrombotic risk in protein S deficiency: the PROSIT study. Hum Mutat 25(3):259\u2013269. https:\/\/doi.org\/10.1002\/humu.20136","journal-title":"Hum Mutat"},{"issue":"16","key":"5607_CR19","doi-asserted-by":"publisher","first-page":"1222","DOI":"10.1056\/NEJM200104193441607","volume":"344","author":"U Seligsohn","year":"2001","unstructured":"Seligsohn U, Lubetsky A (2001) Genetic susceptibility to venous thrombosis. N Engl J Med 344(16):1222\u20131231. https:\/\/doi.org\/10.1056\/NEJM200104193441607","journal-title":"N Engl J Med"},{"issue":"6","key":"5607_CR20","doi-asserted-by":"publisher","first-page":"1222","DOI":"10.1111\/j.1365-2516.2008.01775.x","volume":"14","author":"MK ten Kate","year":"2008","unstructured":"ten Kate MK, van der Meer J (2008) Protein S deficiency: a clinical perspective. Haemophilia 14(6):1222\u20131228","journal-title":"Haemophilia"},{"issue":"2","key":"5607_CR21","doi-asserted-by":"publisher","first-page":"576","DOI":"10.1093\/cvr\/cvaa044","volume":"117","author":"DM Siegal","year":"2021","unstructured":"Siegal DM, Eikelboom JW et al (2021) Variations in incidence of venous thromboembolism in low-, middle-, and high-income countries. Cardiovasc Res 117(2):576\u2013584. https:\/\/doi.org\/10.1093\/cvr\/cvaa044","journal-title":"Cardiovasc Res"},{"key":"5607_CR22","doi-asserted-by":"publisher","first-page":"787","DOI":"10.1002\/ajh.20689","volume":"81","author":"K Mizukami","year":"2006","unstructured":"Mizukami K, Nakabayashi T, Naitoh S et al (2006) One novel and one recurrent mutation in the PROS1 gene cause type I protein S deficiency in patients with pulmonary embolism associated with deep vein thrombosis. Am J Hematol 81:787\u2013797. https:\/\/doi.org\/10.1002\/ajh.20689","journal-title":"Am J Hematol"},{"issue":"3","key":"5607_CR23","doi-asserted-by":"publisher","first-page":"227","DOI":"10.1177\/0897190014530390","volume":"27","author":"HM Phillippe","year":"2014","unstructured":"Phillippe HM, Hornsby LB, Treadway S et al (2014) Inherited thrombophilia. J Pharm Pract 27(3):227\u2013233. https:\/\/doi.org\/10.1177\/0897190014530390","journal-title":"J Pharm Pract"},{"issue":"1","key":"5607_CR24","doi-asserted-by":"publisher","first-page":"154","DOI":"10.1007\/s11239-015-1316-1","volume":"41","author":"SM Stevens","year":"2016","unstructured":"Stevens SM, Woller SC, Bauer KA et al (2016) Guidance for the evaluation and treatment of hereditary and acquired thrombophilia. J Thromb Thrombolysis 41(1):154\u2013164. https:\/\/doi.org\/10.1007\/s11239-015-1316-1","journal-title":"J Thromb Thrombolysis"},{"issue":"12","key":"5607_CR25","doi-asserted-by":"publisher","first-page":"1069","DOI":"10.1136\/jcp.46.12.1069","volume":"46","author":"BT Colvin","year":"1993","unstructured":"Colvin BT, Machin SJ, Barrowcliffe TW et al (1993) Audit of oral anticoagulant treatment. The BCSH Haemostasis and Thrombosis Task Force of the British Society for Haematology. J Clin Pathol 46(12):1069\u20131070. https:\/\/doi.org\/10.1136\/jcp.46.12.1069","journal-title":"J Clin Pathol"}],"container-title":["Annals of Hematology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1007\/s00277-023-05607-6.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/article\/10.1007\/s00277-023-05607-6\/fulltext.html","content-type":"text\/html","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1007\/s00277-023-05607-6.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2024,1,19]],"date-time":"2024-01-19T07:07:02Z","timestamp":1705648022000},"score":1,"resource":{"primary":{"URL":"https:\/\/link.springer.com\/10.1007\/s00277-023-05607-6"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2024,1,4]]},"references-count":25,"journal-issue":{"issue":"2","published-print":{"date-parts":[[2024,2]]}},"alternative-id":["5607"],"URL":"https:\/\/doi.org\/10.1007\/s00277-023-05607-6","relation":{},"ISSN":["0939-5555","1432-0584"],"issn-type":[{"value":"0939-5555","type":"print"},{"value":"1432-0584","type":"electronic"}],"subject":[],"published":{"date-parts":[[2024,1,4]]},"assertion":[{"value":"15 November 2023","order":1,"name":"received","label":"Received","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"21 December 2023","order":2,"name":"accepted","label":"Accepted","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"4 January 2024","order":3,"name":"first_online","label":"First Online","group":{"name":"ArticleHistory","label":"Article History"}},{"order":1,"name":"Ethics","group":{"name":"EthicsHeading","label":"Declarations"}},{"value":"The authors declare no competing interests.","order":2,"name":"Ethics","group":{"name":"EthicsHeading","label":"Conflict of interest"}}]}}