{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,2,26]],"date-time":"2026-02-26T04:40:27Z","timestamp":1772080827512,"version":"3.50.1"},"reference-count":64,"publisher":"Oxford University Press (OUP)","issue":"4","license":[{"start":{"date-parts":[[2024,11,24]],"date-time":"2024-11-24T00:00:00Z","timestamp":1732406400000},"content-version":"vor","delay-in-days":54,"URL":"https:\/\/creativecommons.org\/licenses\/by-nc\/4.0\/"}],"funder":[{"DOI":"10.13039\/100000050","name":"National Heart, Lung, and Blood Institute","doi-asserted-by":"publisher","award":["HL146508 to M.A."],"award-info":[{"award-number":["HL146508 to M.A."]}],"id":[{"id":"10.13039\/100000050","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100000102","name":"Health Resources and Services Administration","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100000102","id-type":"DOI","asserted-by":"publisher"}]},{"name":"Mayo Clinic Comprehensive Hemophilia Treatment Center","award":["H30MC24052"],"award-info":[{"award-number":["H30MC24052"]}]},{"DOI":"10.13039\/100000057","name":"National Institute of General Medical Sciences","doi-asserted-by":"publisher","award":["GM143949"],"award-info":[{"award-number":["GM143949"]}],"id":[{"id":"10.13039\/100000057","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2024,10,1]]},"abstract":"<jats:title>Abstract<\/jats:title>\n               <jats:p>The A1 domain in Von Willebrand Factor (VWF) initiates coagulation through binding to platelet glycoprotein GPIb\u03b1 receptors. Von Willebrand Disease (VWD)-Mutations in A1 that either impair (type 2M) or enhance (type 2B) platelet adhesion to VWF can locally destabilize and even misfold the domain. We leveraged\u00a0misfolding in the gain-of-function type 2B VWD phenotype as a\u00a0target, distinct from the normal conformation. Two nuclease-resistant 2\u2032-fluoropyrimidine RNA aptamers were selected to discriminate normal A1 domains from a type 2B V1314D A1 variant in a glycosylated A1A2A3 tri-domain VWF-fragment. Two aptamers, W9 and V1, were isolated that selectively recognize, bind, and inhibit the A1\u2013GPIb\u03b1 interaction with\u00a0WT A1A2A3 and V1314D A1A2A3, respectively. These aptamers were tested against their respective recombinant targets, plasma VWF, VWF concentrates, and patient plasma with the heterozygous type 2B VWD R1306W variant using clinical assays, surface plasmon resonance and inhibition assays of platelet adhesion to recombinant A1 and A1A2A3 domains under shear stress. The specificity of W9 and V1 aptamers confirms that pathological conformations of VWD Type 2B proteins are different from normal VWF. The availability of aptamers that distinguish normal plasma-derived VWF from VWD suggests potential applicability in clinical diagnosis of severe gain-of-function phenotypes.<\/jats:p>","DOI":"10.1093\/narmme\/ugae021","type":"journal-article","created":{"date-parts":[[2024,11,24]],"date-time":"2024-11-24T03:46:44Z","timestamp":1732420004000},"source":"Crossref","is-referenced-by-count":1,"title":["Conformation-specific RNA aptamers for phenotypic distinction between normal von Willebrand factor and type 2B von Willebrand disease"],"prefix":"10.1093","volume":"1","author":[{"given":"Venkata\u00a0R","family":"Machha","sequence":"first","affiliation":[{"name":"Division of Hematology, Department of Internal Medicine, Mayo Clinic , 200 1st St SW,\u00a0 Rochester, MN ,","place":["USA"]},{"name":"Department of Biochemistry and Molecular Biology, Mayo Clinic , 200 1st St SW,\u00a0Rochester, MN 55905,","place":["USA"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-9909-5262","authenticated-orcid":false,"given":"Alexander","family":"Tischer","sequence":"additional","affiliation":[{"name":"Division of Hematology, Department of Internal Medicine, Mayo Clinic , 200 1st St SW,\u00a0 Rochester, MN ,","place":["USA"]},{"name":"Department of Biochemistry and Molecular Biology, Mayo Clinic , 200 1st St SW,\u00a0Rochester, MN 55905,","place":["USA"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Laurie","family":"Moon-Tasson","sequence":"additional","affiliation":[{"name":"Division of Hematology, Department of Internal Medicine, Mayo Clinic , 200 1st St SW,\u00a0 Rochester, MN ,","place":["USA"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Julie","family":"Tange","sequence":"additional","affiliation":[{"name":"Special Coagulation Laboratory, Mayo Medical Laboratories, Mayo Clinic , 200 1st St SW,\u00a0Rochester, MN 55905,","place":["USA"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Annyoceli","family":"Santiago-Davis","sequence":"additional","affiliation":[{"name":"Department of Biochemistry and Molecular Biology, Mayo Clinic , 200 1st St SW,\u00a0Rochester, MN 55905,","place":["USA"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Rajiv\u00a0K","family":"Pruthi","sequence":"additional","affiliation":[{"name":"Division of Hematopathology, Division of Laboratory Genetics and Genomics, Department of Laboratory Medicine and Pathology, Mayo Clinic , 200 1st St SW, Rochester, MN 55905,","place":["USA"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-7627-9560","authenticated-orcid":false,"given":"Dong","family":"Chen","sequence":"additional","affiliation":[{"name":"Division of Hematopathology, Department of Laboratory Medicine and Pathology, Mayo Clinic , 200 1st St SW, Rochester, MN 55905,","place":["USA"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-5043-6422","authenticated-orcid":false,"suffix":"III","given":"L James","family":"Maher","sequence":"additional","affiliation":[{"name":"Department of Biochemistry and Molecular Biology, Mayo Clinic , 200 1st St SW,\u00a0Rochester, MN 55905,","place":["USA"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-4446-6932","authenticated-orcid":false,"given":"Matthew","family":"Auton","sequence":"additional","affiliation":[{"name":"Division of Hematology, Department of Internal Medicine, Mayo Clinic , 200 1st St SW,\u00a0 Rochester, MN ,","place":["USA"]},{"name":"Department of Biochemistry and Molecular Biology, Mayo Clinic , 200 1st St SW,\u00a0Rochester, MN 55905,","place":["USA"]}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"286","published-online":{"date-parts":[[2024,11,23]]},"reference":[{"key":"2025010217145552900_B1","doi-asserted-by":"crossref","first-page":"324","DOI":"10.1038\/s41467-017-00230-2","article-title":"Flow-induced elongation of von Willebrand factor precedes tension-dependent activation","volume":"8","author":"Fu","year":"2017","journal-title":"Nat. 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