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Naslov:Evidence of B cell clonality and investigation into properties of the IgM in patients with Schnitzler syndrome
Avtorji:Pathak, Shelly (Avtor)
Rowczenio, Dorota (Avtor)
Lara-Reyna, Samuel (Avtor)
Kačar, Mark (Avtor)
Owen, Roger (Avtor)
Doody, Gina (Avtor)
Krause, Karoline (Avtor)
Lachmann, Helen J (Avtor)
Doffinger, Rainer (Avtor)
Newton, Darren (Avtor)
Savic, Sinisa (Avtor)
Jezik:Angleški jezik
Tipologija:1.01 - Izvirni znanstveni članek
Organizacija:Logo UKPBAG - Univerzitetna klinika za pljučne bolezni in alergijo Golnik
Povzetek:The Schnitzler Syndrome (SchS) is an acquired, autoinflammatory condition successfully treated with IL-1 inhibition. The two main defining features of this late-onset condition are neutrophilic urticarial dermatoses (NUD) and the presence of an IgM monoclonal component. While the former aspect has been extensively studied in this disease setting, the enigmatic paraproteinaemia and its potential consequential effects within SchS, has not previously been thoroughly addressed. Previous studies analyzing clonal B cell repertoires have largely focused on autoimmune disorders such as Systemic Lupus Erythematous (SLE) and hematological malignancies such as Chronic Lymphocytic Leukaemia (CLL), where B-cell clonality is central to disease pathology. The present study uses next-generation sequencing to provide detailed insight into aspects of B cell VDJ recombination and properties of the resulting immunoglobulin chains. An overview of IgH regional dynamics in 10 SchS patients, with a particular focus on CDR3 sequences and VDJ gene usage is reported, highlighting the presence of specific B cell expansions. Protein microarray detected a substantial proportion of autoreactive IgM to nuclear target proteins, though a single universal target was not identified. Together, these genetic and functional findings impart new understanding into this rare disorder.
Ključne besede:Schnitzler syndrome, B-lymhocytes, paraproteinemias, pararoteins, immunoglobulin M, autoinflammatory diseases, IgM
Leto izida:2020
Založnik:Frontiers Media SA
Izvor:Švica
UDK:616-097
ISSN pri članku:1664-3224
COBISS_ID:58382339 Povezava se odpre v novem oknu
DOI:10.3389/fimmu.2020.569006 Povezava se odpre v novem oknu
Opombe:Nasl. z nasl. zaslona; Soavtor iz Slovenije: Mark Kačar; Opis vira z dne 6. 4. 2021; Št. članka: 569006;
Število ogledov:3
Število prenosov:1
Datoteke:URL URL - Predstavitvena datoteka, za dostop obiščite https://www.frontiersin.org/articles/10.3389/fimmu.2020.569006/pdf
 
Nadgradivo:Front. immunol.
Frontiers Research Foundation
 
Metapodatki:XML RDF-CHPDL DC-XML DC-RDF
Avtorske pravice: © 2020 Pathak, Rowczenio, Lara-Reyna, Kacar, Owen, Doody, Krause, Lachmann, Doffinger, Newton and Savic.
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Licence

Licenca:CC BY 4.0, Creative Commons Priznanje avtorstva 4.0 Mednarodna
Povezava:http://creativecommons.org/licenses/by/4.0/deed.sl
Opis:To je standardna licenca Creative Commons, ki daje uporabnikom največ možnosti za nadaljnjo uporabo dela, pri čemer morajo navesti avtorja.
Začetek licenciranja:03.12.2020

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