FRINK Linked Data Fragments server

Matches in SemOpenAlex for { <https://semopenalex.org/work/W1900104791> ?p ?o ?g. }

• W1900104791 endingPage "3146" @default.
• W1900104791 startingPage "3132" @default.
• W1900104791 abstract "Hereditary angioedema type III (HAEIII) is a rare inherited swelling disorder that is associated with point mutations in the gene encoding the plasma protease factor XII (FXII). Here, we demonstrate that HAEIII-associated mutant FXII, derived either from HAEIII patients or recombinantly produced, is defective in mucin-type Thr309-linked glycosylation. Loss of glycosylation led to increased contact-mediated autoactivation of zymogen FXII, resulting in excessive activation of the bradykinin-forming kallikrein-kinin pathway. In contrast, both FXII-driven coagulation and the ability of C1-esterase inhibitor to bind and inhibit activated FXII were not affected by the mutation. Intravital laser-scanning microscopy revealed that, compared with control animals, both F12-/- mice reconstituted with recombinant mutant forms of FXII and humanized HAEIII mouse models with inducible liver-specific expression of Thr309Lys-mutated FXII exhibited increased contact-driven microvascular leakage. An FXII-neutralizing antibody abolished bradykinin generation in HAEIII patient plasma and blunted edema in HAEIII mice. Together, the results of this study characterize the mechanism of HAEIII and establish FXII inhibition as a potential therapeutic strategy to interfere with excessive vascular leakage in HAEIII and potentially alleviate edema due to other causes." @default.
• W1900104791 created "2016-06-24" @default.
• W1900104791 creator A5006168170 @default.
• W1900104791 creator A5008405084 @default.
• W1900104791 creator A5015347425 @default.
• W1900104791 creator A5020006815 @default.
• W1900104791 creator A5023752156 @default.
• W1900104791 creator A5035337724 @default.
• W1900104791 creator A5037913819 @default.
• W1900104791 creator A5041800520 @default.
• W1900104791 creator A5043502169 @default.
• W1900104791 creator A5045365245 @default.
• W1900104791 creator A5052804595 @default.
• W1900104791 creator A5060939250 @default.
• W1900104791 creator A5079831221 @default.
• W1900104791 creator A5086660690 @default.
• W1900104791 date "2015-07-20" @default.
• W1900104791 modified "2023-10-14" @default.
• W1900104791 title "Defective glycosylation of coagulation factor XII underlies hereditary angioedema type III" @default.
• W1900104791 cites W1485381400 @default.
• W1900104791 cites W1522956225 @default.
• W1900104791 cites W1543284954 @default.
• W1900104791 cites W1556501884 @default.
• W1900104791 cites W1564822583 @default.
• W1900104791 cites W1947012397 @default.
• W1900104791 cites W1971042333 @default.
• W1900104791 cites W1973740388 @default.
• W1900104791 cites W1981415884 @default.
• W1900104791 cites W1982170684 @default.
• W1900104791 cites W1983010160 @default.
• W1900104791 cites W1983969090 @default.
• W1900104791 cites W1986207725 @default.
• W1900104791 cites W1987529569 @default.
• W1900104791 cites W1988437478 @default.
• W1900104791 cites W1992251016 @default.
• W1900104791 cites W1993916452 @default.
• W1900104791 cites W1996136104 @default.
• W1900104791 cites W1998811679 @default.
• W1900104791 cites W2003266341 @default.
• W1900104791 cites W2012160462 @default.
• W1900104791 cites W2014314228 @default.
• W1900104791 cites W2020160101 @default.
• W1900104791 cites W2024545582 @default.
• W1900104791 cites W2025267980 @default.
• W1900104791 cites W2027158958 @default.
• W1900104791 cites W2036655503 @default.
• W1900104791 cites W2047425163 @default.
• W1900104791 cites W2048115021 @default.
• W1900104791 cites W2059684863 @default.
• W1900104791 cites W2064514230 @default.
• W1900104791 cites W2065599201 @default.
• W1900104791 cites W2067354269 @default.
• W1900104791 cites W2072242577 @default.
• W1900104791 cites W2074269298 @default.
• W1900104791 cites W2077024547 @default.
• W1900104791 cites W2078094108 @default.
• W1900104791 cites W2079221176 @default.
• W1900104791 cites W2081334010 @default.
• W1900104791 cites W2081848037 @default.
• W1900104791 cites W2083884362 @default.
• W1900104791 cites W2085233691 @default.
• W1900104791 cites W2089961321 @default.
• W1900104791 cites W2092800556 @default.
• W1900104791 cites W2093701093 @default.
• W1900104791 cites W2099127967 @default.
• W1900104791 cites W2105301485 @default.
• W1900104791 cites W2113720308 @default.
• W1900104791 cites W2115565372 @default.
• W1900104791 cites W2125474642 @default.
• W1900104791 cites W2127370427 @default.
• W1900104791 cites W2128921599 @default.
• W1900104791 cites W2129582005 @default.
• W1900104791 cites W2134114878 @default.
• W1900104791 cites W2134463696 @default.
• W1900104791 cites W2142964320 @default.
• W1900104791 cites W2143914653 @default.
• W1900104791 cites W2149853382 @default.
• W1900104791 cites W2151429375 @default.
• W1900104791 cites W2152338078 @default.
• W1900104791 cites W2163505092 @default.
• W1900104791 cites W2164980913 @default.
• W1900104791 cites W2166268582 @default.
• W1900104791 cites W2228741158 @default.
• W1900104791 cites W2276599878 @default.
• W1900104791 cites W2312544893 @default.
• W1900104791 cites W2313107232 @default.
• W1900104791 cites W2330698677 @default.
• W1900104791 cites W2340244022 @default.
• W1900104791 cites W2413038113 @default.
• W1900104791 cites W247310062 @default.
• W1900104791 cites W50187013 @default.
• W1900104791 doi "https://doi.org/10.1172/jci77139" @default.
• W1900104791 hasPubMedCentralId "https://www.ncbi.nlm.nih.gov/pmc/articles/4563738" @default.
• W1900104791 hasPubMedId "https://pubmed.ncbi.nlm.nih.gov/26193639" @default.
• W1900104791 hasPublicationYear "2015" @default.
• W1900104791 type Work @default.
• W1900104791 sameAs 1900104791 @default.
• W1900104791 citedByCount "136" @default.

• next