{"database":"biostudies-literature","file_versions":[],"scores":null,"additional":{"omics_type":["Unknown"],"volume":["9(20)"],"submitter":["Postmus T"],"pubmed_abstract":["<h4>Abstract</h4>Immune-mediated thrombotic thrombocytopenic purpura (iTTP) is a rare life-threatening thrombotic disorder, which results from the development of autoantibodies targeting ADAMTS13. Most patients (>90%) with iTTP display antibodies against a shared epitope in the spacer domain of ADAMTS13. Nevertheless, a smaller population of patients (20%-40%) also has antibodies directed toward the CUB (complement C1r/C1s, Uegf, Bmp1) domains of ADAMTS13. Here, we explored whether anti-CUB antibodies have a shared epitope located on CUB domains of ADAMTS13 that overlaps with the spacer-CUB domain interface. Hydrogen-deuterium exchange mass spectrometry revealed that a panel of patient-derived human monoclonal anti-CUB domain antibodies specifically targeted peptides 1248-1253 and 1359-137"],"journal":["Blood advances"],"pagination":["5350-5361"],"full_dataset_link":["https://www.ebi.ac.uk/biostudies/studies/S-EPMC12597635"],"repository":["biostudies-literature"],"pubmed_title":["Trp1250, Lys1252, and Arg1367 of ADAMTS13 comprise a hot spot for anti-CUB domain antibodies in patients with iTTP."],"pmcid":["PMC12597635"],"pubmed_authors":["Hoogendijk AJ","van Alphen F","Postmus T","Kaijen P","Veyradier A","Coppo P","van der Zwaan C","Vanhoorelbeke K","Nicolaes G","Jansen R","Voorberg J","Joly BS"],"additional_accession":[]},"is_claimable":false,"name":"Trp1250, Lys1252, and Arg1367 of ADAMTS13 comprise a hot spot for anti-CUB domain antibodies in patients with iTTP.","description":"<h4>Abstract</h4>Immune-mediated thrombotic thrombocytopenic purpura (iTTP) is a rare life-threatening thrombotic disorder, which results from the development of autoantibodies targeting ADAMTS13. Most patients (>90%) with iTTP display antibodies against a shared epitope in the spacer domain of ADAMTS13. Nevertheless, a smaller population of patients (20%-40%) also has antibodies directed toward the CUB (complement C1r/C1s, Uegf, Bmp1) domains of ADAMTS13. Here, we explored whether anti-CUB antibodies have a shared epitope located on CUB domains of ADAMTS13 that overlaps with the spacer-CUB domain interface. Hydrogen-deuterium exchange mass spectrometry revealed that a panel of patient-derived human monoclonal anti-CUB domain antibodies specifically targeted peptides 1248-1253 and 1359-137","dates":{"release":"2025-01-01T00:00:00Z","publication":"2025 Oct","modification":"2026-06-05T12:23:15.263Z","creation":"2026-05-16T03:12:34.133Z"},"accession":"S-EPMC12597635","cross_references":{"pubmed":["40779569"],"doi":["10.1182/bloodadvances.2025017193"]}}