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000268464 1001_ $$00000-0002-9092-1684$$aWolf, Christine$$b0
000268464 245__ $$aUNC93B1 variants underlie TLR7-dependent autoimmunity.
000268464 260__ $$aWashington, DC$$bAAAS$$c2024
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000268464 520__ $$aUNC93B1 is critical for trafficking and function of nucleic acid-sensing Toll-like receptors (TLRs) TLR3, TLR7, TLR8, and TLR9, which are essential for antiviral immunity. Overactive TLR7 signaling induced by recognition of self-nucleic acids has been implicated in systemic lupus erythematosus (SLE). Here, we report UNC93B1 variants (E92G and R336L) in four patients with early-onset SLE. Patient cells or mouse macrophages carrying the UNC93B1 variants produced high amounts of TNF-α and IL-6 and upon stimulation with TLR7/TLR8 agonist, but not with TLR3 or TLR9 agonists. E92G causes UNC93B1 protein instability and reduced interaction with TLR7, leading to selective TLR7 hyperactivation with constitutive type I IFN signaling. Thus, UNC93B1 regulates TLR subtype-specific mechanisms of ligand recognition. Our findings establish a pivotal role for UNC93B1 in TLR7-dependent autoimmunity and highlight the therapeutic potential of targeting TLR7 in SLE.
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000268464 650_7 $$2NLM Chemicals$$aToll-Like Receptor 7
000268464 650_7 $$2NLM Chemicals$$aToll-Like Receptor 9
000268464 650_7 $$2NLM Chemicals$$aToll-Like Receptor 8
000268464 650_7 $$2NLM Chemicals$$aToll-Like Receptor 3
000268464 650_7 $$2NLM Chemicals$$aTLR7 protein, human
000268464 650_7 $$2NLM Chemicals$$aUNC93B1 protein, human
000268464 650_7 $$2NLM Chemicals$$aMembrane Transport Proteins
000268464 650_7 $$2NLM Chemicals$$aUNC93B1 protein, mouse
000268464 650_2 $$2MeSH$$aMice
000268464 650_2 $$2MeSH$$aAnimals
000268464 650_2 $$2MeSH$$aHumans
000268464 650_2 $$2MeSH$$aToll-Like Receptor 7: genetics
000268464 650_2 $$2MeSH$$aAutoimmunity: genetics
000268464 650_2 $$2MeSH$$aToll-Like Receptor 9: metabolism
000268464 650_2 $$2MeSH$$aToll-Like Receptor 8
000268464 650_2 $$2MeSH$$aToll-Like Receptor 3: metabolism
000268464 650_2 $$2MeSH$$aLupus Erythematosus, Systemic: genetics
000268464 650_2 $$2MeSH$$aMembrane Transport Proteins
000268464 7001_ $$00000-0002-5173-2979$$aLim, Ee Lyn$$b1
000268464 7001_ $$00000-0003-0658-2147$$aMokhtari, Mohammad$$b2
000268464 7001_ $$00009-0002-5254-6411$$aKind, Barbara$$b3
000268464 7001_ $$00000-0002-2806-6904$$aOdainic, Alexandru$$b4
000268464 7001_ $$aLara-Villacanas, Eusebia$$b5
000268464 7001_ $$aKoss, Sarah$$b6
000268464 7001_ $$00000-0003-1447-6811$$aMages, Simon$$b7
000268464 7001_ $$00009-0001-8558-3692$$aMenzel, Katharina$$b8
000268464 7001_ $$aEngel, Kerstin$$b9
000268464 7001_ $$aDückers, Gregor$$b10
000268464 7001_ $$aBernbeck, Benedikt$$b11
000268464 7001_ $$00000-0001-8153-1601$$aSchneider, Dominik T$$b12
000268464 7001_ $$aSiepermann, Kathrin$$b13
000268464 7001_ $$00000-0002-9945-8833$$aNiehues, Tim$$b14
000268464 7001_ $$00000-0003-3407-7631$$aGoetzke, Carl Christoph$$b15
000268464 7001_ $$00000-0002-6179-0670$$aDurek, Pawel$$b16
000268464 7001_ $$aMinden, Kirsten$$b17
000268464 7001_ $$00000-0002-6478-7725$$aDörner, Thomas$$b18
000268464 7001_ $$00000-0002-0500-0304$$aStittrich, Anna$$b19
000268464 7001_ $$00000-0001-7850-5971$$aSzelinski, Franziska$$b20
000268464 7001_ $$00000-0002-9773-4929$$aGuerra, Gabriela Maria$$b21
000268464 7001_ $$aMassoud, Mona$$b22
000268464 7001_ $$00000-0002-5561-3635$$aBieringer, Markus$$b23
000268464 7001_ $$00000-0002-1852-9880$$ade Oliveira Mann, Carina C$$b24
000268464 7001_ $$00000-0002-7266-4098$$aBeltrán, Eduardo$$b25
000268464 7001_ $$00000-0003-2404-9397$$aKallinich, Tilmann$$b26
000268464 7001_ $$00000-0002-8015-6907$$aMashreghi, Mir-Farzin$$b27
000268464 7001_ $$00000-0001-9561-2752$$aSchmidt, Susanne V$$b28
000268464 7001_ $$0P:(DE-2719)2000062$$aLatz, Eicke$$b29$$udzne
000268464 7001_ $$00000-0002-3628-9278$$aKlughammer, Johanna$$b30
000268464 7001_ $$00000-0001-9467-4898$$aMajer, Olivia$$b31
000268464 7001_ $$00000-0002-4008-9869$$aLee-Kirsch, Min Ae$$b32
000268464 773__ $$0PERI:(DE-600)2862556-0$$a10.1126/sciimmunol.adi9769$$gVol. 9, no. 92, p. eadi9769$$n92$$peadi9769$$tScience immunology$$v9$$x2470-9468$$y2024
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