| Large-scale mutational analysis identifies UNC93B1 variants that drive TLR-mediated autoimmunity in mice and humans. | Large-scale mutational analysis identifies UNC93B1 variants that drive TLR-mediated autoimmunity in mice and humans.
Rael VE, Yano JA, Huizar JP, Slayden LC, Weiss MA, Turcotte EA, Terry JM, Zuo W, Thiffault I, Pastinen T, Farrow EG, Jenkins JL, Becker ML, Wong SC, Stevens AM, Otten C, Allenspach EJ, Bonner DE, Bernstein JA, Wheeler MT, Saxton RA, Undiagnosed Diseases Network, Liu B, Majer O, Barton GM., Free PMC Article | 09/4/2024 |
| Gain-of-function human UNC93B1 variants cause systemic lupus erythematosus and chilblain lupus. | Gain-of-function human UNC93B1 variants cause systemic lupus erythematosus and chilblain lupus.
David C, Arango-Franco CA, Badonyi M, Fouchet J, Rice GI, Didry-Barca B, Maisonneuve L, Seabra L, Kechiche R, Masson C, Cobat A, Abel L, Talouarn E, Béziat V, Deswarte C, Livingstone K, Paul C, Malik G, Ross A, Adam J, Walsh J, Kumar S, Bonnet D, Bodemer C, Bader-Meunier B, Marsh JA, Casanova JL, Crow YJ, Manoury B, Frémond ML, Bohlen J, Lepelley A., Free PMC Article | 06/27/2024 |
| Genetic variants in UNC93B1 predispose to childhood-onset systemic lupus erythematosus. | Genetic variants in UNC93B1 predispose to childhood-onset systemic lupus erythematosus.
Al-Azab M, Idiiatullina E, Liu Z, Lin M, Hrovat-Schaale K, Xian H, Zhu J, Yang M, Lu B, Zhao Z, Liu Y, Chang J, Li X, Guo C, Liu Y, Wu Q, Chen J, Lan C, Zeng P, Cui J, Gao X, Zhou W, Zhang Y, Zhang Y, Masters SL., Free PMC Article | 06/11/2024 |
| UNC93B1 variants underlie TLR7-dependent autoimmunity. | UNC93B1 variants underlie TLR7-dependent autoimmunity.
Wolf C, Lim EL, Mokhtari M, Kind B, Odainic A, Lara-Villacanas E, Koss S, Mages S, Menzel K, Engel K, Dückers G, Bernbeck B, Schneider DT, Siepermann K, Niehues T, Goetzke CC, Durek P, Minden K, Dörner T, Stittrich A, Szelinski F, Guerra GM, Massoud M, Bieringer M, de Oliveira Mann CC, Beltrán E, Kallinich T, Mashreghi MF, Schmidt SV, Latz E, Klughammer J, Majer O, Lee-Kirsch MA. | 02/26/2024 |
| UNC93B1 attenuates the cGAS-STING signaling pathway by targeting STING for autophagy-lysosome degradation. | UNC93B1 attenuates the cGAS-STING signaling pathway by targeting STING for autophagy-lysosome degradation.
Zhu H, Zhang R, Yi L, Tang YD, Zheng C. | 07/23/2022 |
| Dynamic control of nucleic-acid-sensing Toll-like receptors by the endosomal compartment. | Dynamic control of nucleic-acid-sensing Toll-like receptors by the endosomal compartment.
Miyake K, Saitoh SI, Fukui R, Shibata T, Sato R, Murakami Y. | 03/26/2022 |
| Variants Affecting the C-Terminal Tail of UNC93B1 Are Not a Common Risk Factor for Systemic Lupus Erythematosus. | Variants Affecting the C-Terminal Tail of UNC93B1 Are Not a Common Risk Factor for Systemic Lupus Erythematosus.
Kiener S, Ribi C, Keller I, Chizzolini C, Trendelenburg M, Huynh-Do U, von Kempis J, On Behalf Of Swiss Sle Cohort Study Sscs, Leeb T., Free PMC Article | 02/5/2022 |
| Donor UNC-93 Homolog B1 genetic polymorphism predicts survival outcomes after unrelated bone marrow transplantation. | Donor UNC-93 Homolog B1 genetic polymorphism predicts survival outcomes after unrelated bone marrow transplantation.
Uchino K, Vu Quang L, Mizuno S, Horio T, Yamamoto H, Hanamura I, Kodera Y, Luis Espinoza J, Onizuka M, Kashiwase K, Morishima Y, Fukuda T, Doki N, Miyamura K, Mori T, Morishita E, Nakao S, Takami A., Free PMC Article | 01/22/2022 |
| UNC93B1 curbs cytosolic DNA signaling by promoting STING degradation. | UNC93B1 curbs cytosolic DNA signaling by promoting STING degradation.
He Z, Ye S, Xing Y, Jiu Y, Zhong J. | 10/2/2021 |
| Cryo-EM structures of Toll-like receptors in complex with UNC93B1. | Cryo-EM structures of Toll-like receptors in complex with UNC93B1.
Ishida H, Asami J, Zhang Z, Nishizawa T, Shigematsu H, Ohto U, Shimizu T. | 04/3/2021 |
| UNC93B1 regulates cell-cycle arrest at the G1 phase in oral squamous cell carcinoma. Down-regulated UNC93B1 stops granulocyte macrophage colony-stimulating factor. UNC93B1 may control tumors via granulocyte macrophage colony-stimulating factor. | UNC93B1 promotes tumoral growth by controlling the secretion level of granulocyte macrophage colony-stimulating factor in human oral cancer.
Wagai S, Kasamatsu A, Iyoda M, Hayashi F, Hiroshima K, Yoshimura S, Miyamoto I, Nakashima D, Endo-Sakamoto Y, Shiiba M, Tanzawa H, Uzawa K. | 03/28/2020 |
| Our results suggest that rare protein-altering variants in the C10orf88 and UNC93B1 genes are associated with a worse response to anti-VEGF therapy in patients with neovascular age-related macular degeneration, but these results require further validation in other cohorts. | Association of Genetic Variants With Response to Anti-Vascular Endothelial Growth Factor Therapy in Age-Related Macular Degeneration.
Lorés-Motta L, Riaz M, Grunin M, Corominas J, van Asten F, Pauper M, Leenders M, Richardson AJ, Muether P, Cree AJ, Griffiths HL, Pham C, Belanger MC, Meester-Smoor MA, Ali M, Heid IM, Fritsche LG, Chakravarthy U, Gale R, McKibbin M, Inglehearn CF, Schlingemann RO, Omar A, Chen J, Koenekoop RK, Fauser S, Guymer RH, Hoyng CB, de Jong EK, Lotery AJ, Mitchell P, den Hollander AI, Baird PN, Chowers I., Free PMC Article | 10/12/2019 |
| Expression of a constitutively active STIM1 mutant, which no longer binds UNC93B1, restores antigen degradation and cross-presentation in 3d-mutated dendritic cells. | UNC93B1 interacts with the calcium sensor STIM1 for efficient antigen cross-presentation in dendritic cells.
Maschalidi S, Nunes-Hasler P, Nascimento CR, Sallent I, Lannoy V, Garfa-Traore M, Cagnard N, Sepulveda FE, Vargas P, Lennon-Duménil AM, van Endert P, Capiod T, Demaurex N, Darrasse-Jèze G, Manoury B., Free PMC Article | 10/6/2018 |
| Endosomal localization of endogenous TLR3 was decreased by silencing of LRRC59, suggesting that LRRC59 promotes UNC93B1-mediated translocation of NA-sensing TLRs from the ER upon infection. | LRRC59 Regulates Trafficking of Nucleic Acid-Sensing TLRs from the Endoplasmic Reticulum via Association with UNC93B1.
Tatematsu M, Funami K, Ishii N, Seya T, Obuse C, Matsumoto M. | 02/27/2016 |
| the UNC93B1 tyrosine-based motif regulates trafficking and TLR responses via separate mechanisms. | Cutting edge: the UNC93B1 tyrosine-based motif regulates trafficking and TLR responses via separate mechanisms.
Pelka K, Phulphagar K, Zimmermann J, Stahl R, Schmid-Burgk JL, Schmidt T, Spille JH, Labzin LI, Agrawal S, Kandimalla ER, Casanova JL, Hornung V, Marshak-Rothstein A, Höning S, Latz E., Free PMC Article | 11/22/2014 |
| IgM(+)IgD(+)CD27(+) but not switched B cells were strongly reduced in MYD88-, IRAK-4-, and TIRAP-deficient patients, but not UNC-93B-deficient patients. | IgM+IgD+CD27+ B cells are markedly reduced in IRAK-4-, MyD88-, and TIRAP- but not UNC-93B-deficient patients.
Weller S, Bonnet M, Delagreverie H, Israel L, Chrabieh M, Maródi L, Rodriguez-Gallego C, Garty BZ, Roifman C, Issekutz AC, Zitnik SE, Hoarau C, Camcioglu Y, Vasconcelos J, Rodrigo C, Arkwright PD, Cerutti A, Meffre E, Zhang SY, Alcais A, Puel A, Casanova JL, Picard C, Weill JC, Reynaud CA., Free PMC Article | 03/2/2013 |
| TLR3 is the important regulator of UNC93B1 that in turn governs the responsiveness of all TLR3 as the important regulator of UNC93B1 that in turn governs the responsiveness of all NAS Toll-like receptors | The role of UNC93B1 protein in surface localization of TLR3 receptor and in cell priming to nucleic acid agonists.
Pohar J, Pirher N, Benčina M, Manček-Keber M, Jerala R., Free PMC Article | 03/2/2013 |
| UNC93B1 expression is required for TLR3 cleavage and signaling. | Cleavage of Toll-like receptor 3 by cathepsins B and H is essential for signaling.
Garcia-Cattaneo A, Gobert FX, Müller M, Toscano F, Flores M, Lescure A, Del Nery E, Benaroch P., Free PMC Article | 09/1/2012 |
| UNC93B1 physically associates with human TLR8 and regulates TLR8-mediated signaling | UNC93B1 physically associates with human TLR8 and regulates TLR8-mediated signaling.
Itoh H, Tatematsu M, Watanabe A, Iwano K, Funami K, Seya T, Matsumoto M., Free PMC Article | 07/21/2012 |
| To date only 2 children with UNC-93B deficiencies have been identified after isolated HSV-1 encephalitis. | Is it time to study TLR3 or UNC-93B pathway deficiencies in reactivated herpes simplex encephalitis?
Gonzalez-Granado LI. | 07/16/2011 |
| Individuals with congenital mutations develop severe HSV-1 encephalitis | Crosstalk between components of the innate immune system: promoting anti-microbial defenses and avoiding immunopathologies.
Crozat K, Vivier E, Dalod M. | 01/21/2010 |
| regulates ligand-induced trafficking of TLR7 and TLR9 from the ER to endolysosomes, potential therapeutic target for controlling dysregulated TLR7/9 responses in autoimmune diseases | Regulatory molecules required for nucleotide-sensing Toll-like receptors.
Saitoh S, Miyake K. | 01/21/2010 |
| IRAK-4-, MyD88-, and UNC-93B-deficient patients did not display autoreactive antibodies in their serum or develop autoimmune diseases, suggesting that IRAK-4, MyD88, and UNC-93B pathway blockade may thwart autoimmunity in humans. | IRAK-4- and MyD88-dependent pathways are essential for the removal of developing autoreactive B cells in humans.
Isnardi I, Ng YS, Srdanovic I, Motaghedi R, Rudchenko S, von Bernuth H, Zhang SY, Puel A, Jouanguy E, Picard C, Garty BZ, Camcioglu Y, Doffinger R, Kumararatne D, Davies G, Gallin JI, Haraguchi S, Day NK, Casanova JL, Meffre E., Free PMC Article | 01/21/2010 |
| No UNC-93B1 mutations were foundin patients with MRS. | Melkersson-Rosenthal syndrome.
Kanerva M, Moilanen K, Virolainen S, Vaheri A, Pitkäranta A. | 01/21/2010 |
| study confirms the function of UNC-93B for innate immunity in human beings, and extends the knowledge for this molecule to the analysis of its regulation and the subcellular localization of the endogenous protein | Assessing the function of human UNC-93B in Toll-like receptor signaling and major histocompatibility complex II response.
Koehn J, Huesken D, Jaritz M, Rot A, Zurini M, Dwertmann A, Beutler B, Korthäuer U. | 01/21/2010 |