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• W2144031609 abstract "Mutations in Wiskott-Aldrich syndrome (WAS) protein (WASp), a regulator of actin dynamics in hematopoietic cells, cause WAS, an X-linked primary immunodeficiency characterized by recurrent infections and a marked predisposition to develop autoimmune disorders. The mechanisms that link actin alterations to the autoimmune phenotype are still poorly understood. We show that chronic activation of plasmacytoid dendritic cells (pDCs) and elevated type-I interferon (IFN) levels play a role in WAS autoimmunity. WAS patients display increased expression of type-I IFN genes and their inducible targets, alteration in pDCs numbers, and hyperresponsiveness to TLR9. Importantly, ablating IFN-I signaling in WASp null mice rescued chronic activation of conventional DCs, splenomegaly, and colitis. Using WASp-deficient mice, we demonstrated that WASp null pDCs are intrinsically more responsive to multimeric agonist of TLR9 and constitutively secrete type-I IFN but become progressively tolerant to further stimulation. By acute silencing of WASp and actin inhibitors, we show that WASp-mediated actin polymerization controls intracellular trafficking and compartmentalization of TLR9 ligands in pDCs restraining exaggerated activation of the TLR9-IFN-α pathway. Together, these data highlight the role of actin dynamics in pDC innate functions and imply the pDC-IFN-α axis as a player in the onset of autoimmune phenomena in WAS disease." @default.
• W2144031609 created "2016-06-24" @default.
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• W2144031609 date "2013-01-21" @default.
• W2144031609 modified "2023-10-13" @default.
• W2144031609 title "Wiskott-Aldrich syndrome protein–mediated actin dynamics control type-I interferon production in plasmacytoid dendritic cells" @default.
• W2144031609 cites W1481442945 @default.
• W2144031609 cites W1503550759 @default.
• W2144031609 cites W1519766162 @default.
• W2144031609 cites W1522207028 @default.
• W2144031609 cites W1524321385 @default.
• W2144031609 cites W1538520630 @default.
• W2144031609 cites W1798861522 @default.
• W2144031609 cites W1807797373 @default.
• W2144031609 cites W1906056739 @default.
• W2144031609 cites W1968806966 @default.
• W2144031609 cites W1970396987 @default.
• W2144031609 cites W1972082238 @default.
• W2144031609 cites W1973431560 @default.
• W2144031609 cites W1975630733 @default.
• W2144031609 cites W1975832143 @default.
• W2144031609 cites W1976528130 @default.
• W2144031609 cites W1979238901 @default.
• W2144031609 cites W1979468270 @default.
• W2144031609 cites W1981881077 @default.
• W2144031609 cites W1991752238 @default.
• W2144031609 cites W1993483086 @default.
• W2144031609 cites W1997977259 @default.
• W2144031609 cites W1998793015 @default.
• W2144031609 cites W2001532503 @default.
• W2144031609 cites W2012473400 @default.
• W2144031609 cites W2014304617 @default.
• W2144031609 cites W2015466851 @default.
• W2144031609 cites W2016253390 @default.
• W2144031609 cites W2016447788 @default.
• W2144031609 cites W2016899230 @default.
• W2144031609 cites W2019916852 @default.
• W2144031609 cites W2029022457 @default.
• W2144031609 cites W2039475088 @default.
• W2144031609 cites W2040546543 @default.
• W2144031609 cites W2046413321 @default.
• W2144031609 cites W2049332771 @default.
• W2144031609 cites W2052243935 @default.
• W2144031609 cites W2052521175 @default.
• W2144031609 cites W2053527054 @default.
• W2144031609 cites W2053784823 @default.
• W2144031609 cites W2058129524 @default.
• W2144031609 cites W2063214912 @default.
• W2144031609 cites W2065907531 @default.
• W2144031609 cites W2068112917 @default.
• W2144031609 cites W2070638961 @default.
• W2144031609 cites W2071830659 @default.
• W2144031609 cites W2072522584 @default.
• W2144031609 cites W2079587827 @default.
• W2144031609 cites W2084384009 @default.
• W2144031609 cites W2084951281 @default.
• W2144031609 cites W2085559627 @default.
• W2144031609 cites W2088195013 @default.
• W2144031609 cites W2090099695 @default.
• W2144031609 cites W2091675224 @default.
• W2144031609 cites W2092668505 @default.
• W2144031609 cites W2094746130 @default.
• W2144031609 cites W2098552591 @default.
• W2144031609 cites W2100230706 @default.
• W2144031609 cites W2100382163 @default.
• W2144031609 cites W2100510917 @default.
• W2144031609 cites W2100678593 @default.
• W2144031609 cites W2102037966 @default.
• W2144031609 cites W2106252068 @default.
• W2144031609 cites W2106273198 @default.
• W2144031609 cites W2106566281 @default.
• W2144031609 cites W2116178189 @default.
• W2144031609 cites W2118340386 @default.
• W2144031609 cites W2118481418 @default.
• W2144031609 cites W2121525170 @default.
• W2144031609 cites W2124478712 @default.
• W2144031609 cites W2127533496 @default.
• W2144031609 cites W2128309277 @default.
• W2144031609 cites W2129708174 @default.
• W2144031609 cites W2131350165 @default.
• W2144031609 cites W2133451853 @default.
• W2144031609 cites W2134608330 @default.

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