{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,13]],"date-time":"2026-10-13T21:03:08Z","timestamp":1791925388966,"version":"4.3.4"},"reference-count":203,"publisher":"MDPI AG","issue":"5","license":[{"start":{"date-parts":[[2021,2,28]],"date-time":"2021-02-28T00:00:00Z","timestamp":1614470400000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"DOI":"10.13039\/501100002428","name":"Austrian Science Fund","doi-asserted-by":"publisher","award":["P26079-B13"],"award-info":[{"award-number":["P26079-B13"]}],"id":[{"id":"10.13039\/501100002428","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100002428","name":"Austrian Science Fund","doi-asserted-by":"publisher","award":["P32470-684 B"],"award-info":[{"award-number":["P32470-684 B"]}],"id":[{"id":"10.13039\/501100002428","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100002428","name":"Austrian Science Fund","doi-asserted-by":"publisher","award":["F4701-B20"],"award-info":[{"award-number":["F4701-B20"]}],"id":[{"id":"10.13039\/501100002428","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100002428","name":"Austrian Science Fund","doi-asserted-by":"publisher","award":["F4704-B20"],"award-info":[{"award-number":["F4704-B20"]}],"id":[{"id":"10.13039\/501100002428","id-type":"DOI","asserted-by":"publisher"}]},{"name":"Medical-Scientific Fund of the Mayor of Vienna","award":["19019"],"award-info":[{"award-number":["19019"]}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["IJMS"],"abstract":"<jats:p>Tryptase is a serine protease that is predominantly produced by tissue mast cells (MCs) and stored in secretory granules together with other pre-formed mediators. MC activation, degranulation and mediator release contribute to various immunological processes, but also to several specific diseases, such as IgE-dependent allergies and clonal MC disorders. Biologically active tryptase tetramers primarily derive from the two genes TPSB2 (encoding \u03b2-tryptase) and TPSAB1 (encoding either \u03b1- or \u03b2-tryptase). Based on the most common gene copy numbers, three genotypes, 0\u03b1:4\u03b2, 1\u03b1:3\u03b2 and 2\u03b1:2\u03b2, were defined as \u201ccanonical\u201d. About 4\u20136% of the general population carry germline TPSAB1-\u03b1 copy number gains (2\u03b1:3\u03b2, 3\u03b1:2\u03b2 or more \u03b1-extra-copies), resulting in elevated basal serum tryptase levels. This condition has recently been termed hereditary alpha tryptasemia (H\u03b1T). Although many carriers of H\u03b1T appear to be asymptomatic, a number of more or less specific symptoms have been associated with H\u03b1T. Recent studies have revealed a significantly higher H\u03b1T prevalence in patients with systemic mastocytosis (SM) and an association with concomitant severe Hymenoptera venom-induced anaphylaxis. Moreover, H\u03b1T seems to be more common in idiopathic anaphylaxis and MC activation syndromes (MCAS). Therefore, TPSAB1 genotyping should be included in the diagnostic algorithm in patients with symptomatic SM, severe anaphylaxis or MCAS.<\/jats:p>","DOI":"10.3390\/ijms22052458","type":"journal-article","created":{"date-parts":[[2021,2,28]],"date-time":"2021-02-28T20:43:32Z","timestamp":1614545012000},"page":"2458","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":53,"title":["Genetic Regulation of Tryptase Production and Clinical Impact: Hereditary Alpha Tryptasemia, Mastocytosis and Beyond"],"prefix":"10.3390","volume":"22","author":[{"given":"Bettina","family":"Sprinzl","sequence":"first","affiliation":[{"name":"Ludwig Boltzmann Institute for Hematology and Oncology at the Hanusch Hospital, Center for Medical Genetics, Hanusch Hospital, 1140 Vienna, Austria"},{"name":"Center for Medical Genetics, Hanusch Hospital, 1140 Vienna, Austria"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-0917-4117","authenticated-orcid":false,"given":"Georg","family":"Greiner","sequence":"additional","affiliation":[{"name":"Department of Laboratory Medicine, Medical University of Vienna, 1090 Vienna, Austria"},{"name":"Ludwig Boltzmann Institute for Hematology and Oncology, Medical University of Vienna, 1090 Vienna, Austria"},{"name":"Ihr Labor, Medical Diagnostic Laboratories, 1220 Vienna, Austria"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Goekhan","family":"Uyanik","sequence":"additional","affiliation":[{"name":"Ludwig Boltzmann Institute for Hematology and Oncology at the Hanusch Hospital, Center for Medical Genetics, Hanusch Hospital, 1140 Vienna, Austria"},{"name":"Center for Medical Genetics, Hanusch Hospital, 1140 Vienna, Austria"},{"name":"Medical School, Sigmund Freud Private University, 1020 Vienna, Austria"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-3990-4408","authenticated-orcid":false,"given":"Michel","family":"Arock","sequence":"additional","affiliation":[{"name":"Department of Hematology, APHP, Piti\u00e9-Salp\u00eatri\u00e8re-Charles Foix University Hospital and Sorbonne University, 75013 Paris, France"},{"name":"Centre de Recherche des Cordeliers, INSERM, Sorbonne University, Cell Death and Drug Resistance in Hematological Disorders Team, 75006 Paris, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Torsten","family":"Haferlach","sequence":"additional","affiliation":[{"name":"MLL Munich Leukemia Laboratory, 81377 Munich, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Wolfgang R.","family":"Sperr","sequence":"additional","affiliation":[{"name":"Ludwig Boltzmann Institute for Hematology and Oncology, Medical University of Vienna, 1090 Vienna, Austria"},{"name":"Department of Internal Medicine I, Division of Hematology and Hemostaseology, Medical University of Vienna, 1090 Vienna, Austria"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Peter","family":"Valent","sequence":"additional","affiliation":[{"name":"Ludwig Boltzmann Institute for Hematology and Oncology, Medical University of Vienna, 1090 Vienna, Austria"},{"name":"Department of Internal Medicine I, Division of Hematology and Hemostaseology, Medical University of Vienna, 1090 Vienna, Austria"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-7374-4380","authenticated-orcid":false,"given":"Gregor","family":"Hoermann","sequence":"additional","affiliation":[{"name":"Ludwig Boltzmann Institute for Hematology and Oncology, Medical University of Vienna, 1090 Vienna, Austria"},{"name":"MLL Munich Leukemia Laboratory, 81377 Munich, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2021,2,28]]},"reference":[{"key":"ref_1","first-page":"166","article-title":"Beitr\u00e4ge zur kenntniss der granulirten bindegewebszellen und der eosinophilen leukocythen","volume":"3","author":"Ehrlich","year":"1879","journal-title":"Arch. 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