FRINK Linked Data Fragments server

Matches in SemOpenAlex for { <https://semopenalex.org/work/W2922574819> ?p ?o ?g. }

• W2922574819 endingPage "7624" @default.
• W2922574819 startingPage "7615" @default.
• W2922574819 abstract "Hirschsprung disease (HSCR) is a common cause of intestinal obstruction in the newborn. Hirschsprung-associated enterocolitis (HAEC) is a significant and life-threatening complication of HSCR, affecting up to 60% of patients. Animal models of endothelin receptor B (EdnrB) mutation reliably model human HSCR and HAEC. We previously demonstrated intestinal dysbiosis and a gut-specific deficiency of B-lymphocyte-produced secretory IgA (sIgA), the primary effector molecule of mucosal immunity, in mice with homozygous neural crest cell-conditional deletion of EdnrB (EdnrBNCC-/-). To determine mechanisms for sIgA deficiency, we examined intrinsic and extrinsic aspects of B-lymphocyte development and function. Expression of the endothelin axis components [endothelin-1 (ET-1), endothelin-3 (ET-3), endothelin receptor A (EdnrA), EdnrB] were determined over a developmental time course. B-lymphocyte survival and Ig production were assayed in vitro. Polymeric Ig receptor (pIgR)-mediated IgA transport into the intestinal lumen was interrogated. We found endothelin axis component (EdnrA, EdnrB, ET-1, ET-3) expression in developing extramedullary hematopoietic organs and that some splenic B lymphocytes express EdnrB. Splenic B lymphocytes from EdnrBNCC-/- mice showed no intrinsic defect in survival vs. wild-type (WT) B lymphocytes. In vitro stimulation of splenic B lymphocytes demonstrated decreased IgA, IgG, and IgM production in EdnrBNCC-/-vs. WT mice. Additionally, small intestinal pIgR was decreased ∼50% in EdnrBNCC-/- mice. These results suggest an intrinsic B-lymphocyte defect in antibody production as well as an extrinsic defect in IgA transport in the EdnrBNCC-/- model of HAEC. Our results are consistent with human HAEC observations of decreased luminal sIgA and mouse models of other inflammatory bowel diseases, in which decreased pIgR is seen in concert with a dysregulated microbiota. Finally, our results suggest targeting the dysbiotic microbiome and pIgR-mediated sIgA transport as potential therapeutic approaches in prevention and treatment of HAEC.-Medrano, G., Cailleux, F., Guan, P., Kuruvilla, K., Barlow-Anacker, A. J., Gosain, A. B-lymphocyte-intrinsic and -extrinsic defects in secretory immunoglobulinA production in the neural crest-conditional deletion of endothelin receptor B model of Hirschsprung-associated enterocolitis." @default.
• W2922574819 created "2019-04-01" @default.
• W2922574819 creator A5022945814 @default.
• W2922574819 creator A5049086522 @default.
• W2922574819 creator A5049180860 @default.
• W2922574819 creator A5061211562 @default.
• W2922574819 creator A5065960656 @default.
• W2922574819 creator A5088082181 @default.
• W2922574819 date "2019-03-25" @default.
• W2922574819 modified "2023-10-17" @default.
• W2922574819 title "B‐lymphocyte–intrinsic and –extrinsic defects in secretory immunoglobulin A production in the neural crest–conditional deletion of endothelin receptor B model of Hirschsprung‐associated enterocolitis" @default.
• W2922574819 cites W1517833076 @default.
• W2922574819 cites W1564596764 @default.
• W2922574819 cites W1696199194 @default.
• W2922574819 cites W1920779337 @default.
• W2922574819 cites W1926753936 @default.
• W2922574819 cites W1973136410 @default.
• W2922574819 cites W1977275592 @default.
• W2922574819 cites W1983882689 @default.
• W2922574819 cites W1989914533 @default.
• W2922574819 cites W1998356965 @default.
• W2922574819 cites W1998761054 @default.
• W2922574819 cites W2001439220 @default.
• W2922574819 cites W2004413953 @default.
• W2922574819 cites W2005201799 @default.
• W2922574819 cites W2007799047 @default.
• W2922574819 cites W2015015427 @default.
• W2922574819 cites W2028146151 @default.
• W2922574819 cites W2028324999 @default.
• W2922574819 cites W2039676460 @default.
• W2922574819 cites W2045581442 @default.
• W2922574819 cites W2050779257 @default.
• W2922574819 cites W2065174433 @default.
• W2922574819 cites W2069974481 @default.
• W2922574819 cites W2094654299 @default.
• W2922574819 cites W2094667865 @default.
• W2922574819 cites W2103124138 @default.
• W2922574819 cites W2106426059 @default.
• W2922574819 cites W2117266720 @default.
• W2922574819 cites W2124124512 @default.
• W2922574819 cites W2140605673 @default.
• W2922574819 cites W2145237796 @default.
• W2922574819 cites W2170433626 @default.
• W2922574819 cites W2291508529 @default.
• W2922574819 cites W2585400315 @default.
• W2922574819 cites W2588467249 @default.
• W2922574819 cites W2597830263 @default.
• W2922574819 cites W2607156097 @default.
• W2922574819 cites W2738533759 @default.
• W2922574819 cites W2781721033 @default.
• W2922574819 cites W2801111149 @default.
• W2922574819 cites W2803569299 @default.
• W2922574819 cites W2810837823 @default.
• W2922574819 cites W635600514 @default.
• W2922574819 doi "https://doi.org/10.1096/fj.201801913r" @default.
• W2922574819 hasPubMedCentralId "https://www.ncbi.nlm.nih.gov/pmc/articles/6529339" @default.
• W2922574819 hasPubMedId "https://pubmed.ncbi.nlm.nih.gov/30908942" @default.
• W2922574819 hasPublicationYear "2019" @default.
• W2922574819 type Work @default.
• W2922574819 sameAs 2922574819 @default.
• W2922574819 citedByCount "9" @default.
• W2922574819 countsByYear W29225748192019 @default.
• W2922574819 countsByYear W29225748192020 @default.
• W2922574819 countsByYear W29225748192022 @default.
• W2922574819 countsByYear W29225748192023 @default.
• W2922574819 crossrefType "journal-article" @default.
• W2922574819 hasAuthorship W2922574819A5022945814 @default.
• W2922574819 hasAuthorship W2922574819A5049086522 @default.
• W2922574819 hasAuthorship W2922574819A5049180860 @default.
• W2922574819 hasAuthorship W2922574819A5061211562 @default.
• W2922574819 hasAuthorship W2922574819A5065960656 @default.
• W2922574819 hasAuthorship W2922574819A5088082181 @default.
• W2922574819 hasBestOaLocation W29225748191 @default.
• W2922574819 hasConcept C126322002 @default.
• W2922574819 hasConcept C126749454 @default.
• W2922574819 hasConcept C134018914 @default.
• W2922574819 hasConcept C144980905 @default.
• W2922574819 hasConcept C152605822 @default.
• W2922574819 hasConcept C159654299 @default.
• W2922574819 hasConcept C170493617 @default.
• W2922574819 hasConcept C196843134 @default.
• W2922574819 hasConcept C203014093 @default.
• W2922574819 hasConcept C2776258884 @default.
• W2922574819 hasConcept C2777761686 @default.
• W2922574819 hasConcept C2909649922 @default.
• W2922574819 hasConcept C2911175447 @default.
• W2922574819 hasConcept C54355233 @default.
• W2922574819 hasConcept C71924100 @default.
• W2922574819 hasConcept C86803240 @default.
• W2922574819 hasConcept C8891405 @default.
• W2922574819 hasConcept C95444343 @default.
• W2922574819 hasConceptScore W2922574819C126322002 @default.
• W2922574819 hasConceptScore W2922574819C126749454 @default.
• W2922574819 hasConceptScore W2922574819C134018914 @default.
• W2922574819 hasConceptScore W2922574819C144980905 @default.
• W2922574819 hasConceptScore W2922574819C152605822 @default.
• W2922574819 hasConceptScore W2922574819C159654299 @default.
• W2922574819 hasConceptScore W2922574819C170493617 @default.

• next