The Hur Lab
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Publications
2023
A new mode of transcriptional hub assembly by autoimmune regulator Aire
Huoh Y-S., Zhang Q., Torner R., Baca S., Arthanari H. & Hur S. Under review. PDF
Ladder-like multimerization of FoxP3 enables microsatellite recognition and DNA bridging
Zhang W., Leng F., Wang X., Ramirez R., Park J., Benoist C., Hur S. (2023). Nature. PDF
Mutations from patients with IPEX ported to mice reveal different patterns of FoxP3 and Treg dysfunction
Leon J., Chowdhary K., Zhang W., Ramirez RN., André I., Hur S., Mathis D., Benoist C.
(2023). Cell reports (Cambridge), Vol.42 (8), p.113018-113018, Article 113018. PDF
Stress granules are shock absorbers that prevent excessive innate immune responses to dsRNA
Paget M., Cadena C., Ahmad S., Wang H-T., Jordan T., Kim E., Koo B., Lyons S., Ivanov P., tenOever B., Mu X.. and Hur S. (2023). Molecular Cell 83, 1180 - 1196. PDF
2022
ADAR1 prevents autoinflammation by suppressing spontaneous ZBP1 activation
De Reuver, R., Verdonck, S., Dierick, E., Nemegeer, J., Hessmann, E., Ahmad, S., Jans, M., Blancke, G.,
Van Nieuwerburgh, F., Botzki, A., Vereecke, L., Van Loo, G., Declercq, W., Hur S., Vandenabeele, P. & Maelfait, J. (2022). Nature 607, 784 - 789. PDF
Cellular origins of dsRNA, their recognition and consequences.
Chen YG. and Hur S. (2022). Nat Rev Mol Cell Biol. 23(4): 286–301. PDF
FoxP3 can fold into two distinct dimerization states with divergent functional implications for T cell homeostasis.
Leng, F., Zhang, W., Ramirez,R., Leon J., Zhong Y., Van der Veeken J., Rudensky A., Benoist C. and Hur S. (2022). Immunity. PDF
2021
Viral RNA N6-methyladenosine modification modulates both innate and adaptive immune responses of human respiratory syncytial virus.
Xue, M., Zhang, Y., Wang, H., Kairis, L., Lu, M., Ahmad, S., Attia, Z., Harder O., Zhang, Z., Wei, J., Chen, P., Gao, Y., Peeples, M., Sharma, A., Boyaka, P., He, C., Hur, S., Niewiesk, S. and Li, J. (2021). PLOS Pathogens 17(12): e1010142. PDF
Immunogenicity of In Vitro-Transcribed RNA
Xin Mu and Sun Hur (2021). Acc Chem Res 02; 54(21): 4012–4023. PDF
The Role of RNA Editing in the Immune Response
Ahmad, S., Mu, X., & Hur, S. (2021). Methods in molecular biology (Clifton, N.J.), 2181, 287–307. PDF
TLR3 controls constitutive IFN-β antiviral immunity in human fibroblasts and cortical neurons
Gao, D., Ciancanelli, M. J., Zhang, P., Harschnitz, O., Bondet, V., Hasek, M., Chen, J., Mu, X., Itan, Y., Cobat, A., Sancho-Shimizu, V., Bigio, B., Lorenzo, L., Ciceri, G., McAlpine, J., Anguiano, E., Jouanguy, E., Chaussabel, D., Meyts, I., Diamond, M. S., Abel, L., Hur, S., Smith, G., Notarangelo, L., Duffy, D. Studer, L. Casanova, J-L., Zhang, S. Y. (2021). The Journal of clinical investigation, 131(1), e134529. PDF
Death domain fold proteins in immune signaling and transcriptional regulation
Huoh, Y. S., & Hur, S. (2021). The FEBS journal, 10.1111/febs.15901. Advance online publication. PDF
DDX58(RIG-I)-related disease is associated with tissue-specific interferon pathway activation
Prasov, L., Bohnsack, B. L., El Husny, A. S., Tsoi, L. C., Guan, B., Kahlenberg, J. M., Almeida, E., Wang, H., Cowen, E. W., De Jesus, A. A., Jani, P., Billi, A. C., Moroi, S. E., Wasikowski, R., Almeida, I., Almeida, L. N., Kok, F., Garnai, S. J., Mian, S. I., Chen, M. Y., Warner, B., Ferreira, C., Goldbach-Mansky, R., Hur, S., Brooks, B., Richards, J., Hufnagel, R., Gudjonsson, J. E. (2021). Journal of medical genetics, jmedgenet-2020-107447. Advance online publication. PDF
Nonsegmented Negative-Sense RNA Viruses Utilize N6-Methyladenosine (m6A) as a Common Strategy To Evade Host Innate Immunity
Lu, M., Xue, M., Wang, H. T., Kairis, E. L., Ahmad, S., Wei, J., Zhang, Z., Liu, Q., Zhang, Y., Gao, Y., Garcin, D., Peeples, M. E., Sharma, A., Hur, S., He, C., & Li, J. (2021). Journal of virology, 95(9), e01939-20. PDF
Structural analysis of RIG-I-like receptors reveals ancient rules of engagement between diverse RNA helicases and TRIM ubiquitin ligases.
Kato, K., Ahmad, S., Zhu, Z., Young, J. M., Mu, X., Park, S., Malik, H. S., & Hur, S. (2021).Molecular cell,81(3), 599–613.e8. PDF
2020
Substrate recognition by TRIM and TRIM-like proteins in innate immunity
Wang, H. T., & Hur, S. (2020). Seminars in cell & developmental biology, 111, 76–85. PDF
Dual functions of Aire multimerization in the transcriptional regulation of T cell tolerance
Huoh, Y. S., Wu, B., Park, S., Yang, D., Bansal, K., Greenwald, E., Wong, W. P., Mathis, D., & Hur, S. (2020). (1), 1625. PDF
Regulation of cGAS- and RLR-mediated immunity to nucleic acids
Ablasser, A., & Hur, S. (2020). Nature immunology, 21(1), 17–29. PDF
Genetic and phenotypic spectrum associated with IFIH1 gain-of-function
Rice, G., Park, S., Gavazzi, F., Adang, L., Ayuk, L., Van Eyck, L., Seabra, L., Barrea, C., Battini, R., Belot, A., Berg, S., Billette de Villemeur, T., Bley, A., Blumkin, L., Boespflug-Tanguy, O., Briggs, T., Brimble, E., Dale, R., Darin, N., Debray, F-G., De Giorgis, V., Denecke, J., Doummar, D., Drake af Hagelsrum, G., Eleftheriou, D., Estienne, M., Fazzi, E., Feillet, F., Galli, J.,Hartog, N., Harvengt, J., Heron, B., Heron, D., Kelly, D., Lev, D., Levrat, V., Livingston, J., Marti, I., Mignot, C., Mochel, F., Nougues, M-C., Oppermann, I., Pérez-Dueñas, B., Popp, B., Rodero, M., Rodriguez, D., Saletti, V., Sharpe, C., Tonduti, D., Vadlamani, G., Van Haren, K., Tomas Vila, M., Vogt, J., Wassmer, E., Wiedemann, A., Wilson, C., Zerem, A., Zweier, C., Zuberi, S., Orcesi, S., Vanderver, A., Hur, S., Crow, Y. (2020). Human mutation, 41(4), 837–849. PDF
2019
Filament-like assemblies of intracellular nucleic acid sensors: commonalities and differences
Cadena C & Hur S, Mol. Cell, (2019), 76(2): 243-54 (review). PDF
N6-methyladenosine modification controls circular RNA immunity
Chen YG, Chen R, Ahmad S, Verma R, Kasturi S, Amaya L, Broughton JP, Kim J, Cadena C, Pulendran B, Hur S and Chang HY, Mol.Cell (2019). PDF
The FDA-Approved Oral Drug Nitazoxanide Amplifies Host Antiviral Responses and Inhibits Ebola Virus
Jasenosky LD, Cadena C, Mire C, Borisevich, V., Haridas V, Ranjbar S, Nambu, A., Bavari, S., Soloveva, V., Sadukhan, S., Cassell, G., Geisbert, T., Hur, S. and Goldfeld, A. iScience (2019). PDF
Ubiquitin-dependent and -independent roles of E3 ligase RIPLET in innate immunity
Cadena C, Ahmad S, Xavier A, Willemsen J, Park S, Park JW, Oh SW, Fujita T, Hou F, Binder M, & Hur S, Cell, (2019), 177(5):1187-1200. PDF
Double-stranded RNA sensors and modulators in innate immunity
Hur S, Annu. Rev. Immunol, (2019), 37:349-375. PDF
DDX58and Classic Singleton-Merten Syndrome
Ferreira C, Crow YJ, Gahl WA, Gardner P, Goldbach-Mansky R, Hur S, Almeida de Jesus A, Nehrebecky M, Park JW, and Briggs TA,J. of Clinical Immunol.(2019), 39(1):75-80. PDF
2018
Autosomal dominant early-onset spastic paraparesis with brain calcification due to IFIH1 gain-of-function
Ruaud L, Rice GI, Cabrol C, Piard J, Rodero M, van Eyk L, Boucher-Brischoux E, Maertens de Noordhout A, Mare R, Scalais E, Pauly F, Debray FG, Dobyns W, Uggenti C, Park JW, Hur S, Livingston JH Crow YJ, Van Maldergem L, Human Mutation. (2018), 39: 1076-1080. PDF
An origin of the immunogenicity of in vitro transcribed RNA
Mu X, Greenwald E, Ahmad S and Hur S, Nucleic Acid Research, (2018), 46:5239-49. PDF
Breaching self-tolerance to Alu duplex RNA underlies MDA5-mediated inflammation
Ahmad S, Mu X, Yang F, Greenwald E, Park JW, Jacob E, Zhang C-Z and Hur S., Cell, (2018) 172:797-810 --Highlighted in Cell (2018), 172:640-642. PDF
2017
Dominant-negative loss of function arises from a second, more frequent variant within the SAND domain of autoimmune regulator (AIRE).
Abbott JK, Huoh YS, Reynolds P, Yu L, Rewers M, Reddy M, Anderson MS, Hur S, Gelfand EW, J. of Autoimmunity. (2017) 17:30579-6. PDF
Antiviral immunity and Circular RNA: No End in Sight
Cadena C & Hur S, Molecular Cell, (2017) 67(2):163-164 (preview). PDF
Musculoskeletal disease in MDA5-related type I interferonopathy: A mendelian mimic of Jaccud’s Arthropathy
De Carvalho LM, Ehmke N, Park JW, Ngoumou G, Deigendesch N, Kitabayashi N, Melki I, L Souza FF, Tzschach A, Nogueira-Barbosa MH, Ferriani V, Louzada-Junior P, Marques Junior W, Lourenco CM, Horn D, Kallinich T, Stenzel W, Hur S, Rice GI, Crow YJ, Arthritis & Rheumatology. (2017) 69:2081-91. PDF
2016
Filament assemblies in foreign nucleic acid sensors
Sohn J & Hur S, Curr Opin Struct Biol. (2016), 37:134-44 (Review). PDF
Endogenous retroelements and the host innate immune sensors
Mu X, Ahmad S & Hur S, Advances in Immunology, (2016), 132:47-69. PDF
Measuring Monomer-to-Filament Transition of MAVS as an In Vitro Activity Assay for RIG-I-Like Receptors
Wu B, Houh Y & Hur S, Methods Molecular Biology (2016), Vol 1390, In Press, (Book Chapter). PDF
2015
Unusual cutaneous features associated with a heterozygous gain‐of‐function mutation in IFIH1: overlap between Aicardi‐Goutières and Singleton‐Merten syndromes
Bursztejn AC, Briggs TA, Del Toro Duany Y, Anderson BH, O'Sullivan J, Williams SG, Bodemer C, Fraitag S, Gebhard F, Leheup B, Lemelle I, Oojageer A, Raffo E, Schmitt E, Rice GI, Hur S, Crow YJ., Br J Dermatol. (2015) 173:1505-13. PDF
Identification of a Natural Viral RNA Motif That Optimizes Sensing of Viral RNA by RIG-I
Xu J, Mercado-Lopez X, Grier JT, Kim WK, Chun LF, Irvine EB, Del Toro Duany Y, Kell A, Hur S, Gale M Jr, Raj A, Lopez CB. MBio.(2015) 6:e01265-15. PDF
Helicases in antiviral immunity: dual properties as sensors and effectors
Ahmad S and Hur S, Trends in Biochemical Sciences (2015) 40:576-85. (Review). PDF
How RIG-I like receptors activate MAVS
Wu B & Hur S, Curr. Opin. Virol. (2015)12:91-8. PDF
ATP-dependent effector-like functions of RIG-I like receptors
Yao H, Dittmann M, Peisley A, Hoffmann H-H, Gilmore RH, Schmidt T, Schmidt-Burgk J, Hornung V, Rice CM, and Hur S, Molecular Cell, (2015), 58:541-8. --Highlighted in Science Signaling (2015), 8:ec130. PDF
MDA5-filament, dynamics and disease
Del Toro Duany Y, Wu B and Hur S, Curr. Opin. Virol. (2015), 12:20-5 (Review). PDF
2014
Molecular imprinting as a signal activation mechanism of the viral RNA sensor RIG-I
Wu B, Peisley A, Tetrault D, Li Z, Egelman EH, Magor, KE, Walz T, Penczek PA and Hur S, Molecular Cell, (2014), 55: 511-23. Highlighted in Molecular Cell (2014), 55:505-7. PDF
Gain of function mutations in IFIH1 (MDA5) cause a spectrum of human disease phenotypes associated with an upregulation of type I interferon signaling
Rice GI, del Toro Duany Y, Jenkinson EM, Forte GMA, Anderson BH, Ariaudo G, Bader-Meunier B, Baildam EM, Battini R, Beresford MW, Casarano M, Chouchane M, Cimaz R, Collins AE, Cordeiro NJV, Dale RC, Davidson JE, De Waele L, Desguerre I, Faivre L, Fazzi E, Isidor B, Lagae L, Latchman AR, Lebon P, Li C, Livingston JH, Lourenco CM, Mancardi MM, Masurel-Paulet A, McInnes IB, Menezes MP, Mignot C, O’Sullivan J, Orcesi S, Picco PP, Riva E, Robinson RA, Rodrigues D, Salvatici E, Scott C, Szybowska M, Tolmie JL, Vanderver A, Vanhulle C, Vieira JP, Webb K, Whitney RN, Williams SG, Wolfe LA, Zuberi SM, Hur S, Crow YJ. Nature Genetics, (2014), 46:503-9. Highlighted in Nature Genetics (2014), 84:1551-61. PDF
Structural basis for ubiquitin-mediated antiviral signal activation by RIG-I.
Peisley A, Wu B, Xu H, Chen ZJ & Hur S. Nature, (2014), 509:110-4. Highlighted in Cell Research (2014), 24:645-6. PDF
2013
RIG-I forms signaling-competent filaments in an ATP-dependent and ubiquitin-independent manner
Peisley A, Wu B, Yao H, Walz T & Hur S. Molecular Cell, (2013), 51, 573-83, PMID: 23993742. Highlighted in Faculty1000Prime. PDF
Viral counterattack against the host innate immune system
Wu B and Hur S. Cell Research, (2013), 23, 735-6, PMID:23478300 (Review). PDF
Structural Basis for dsRNA recognition, filament formation and antiviral signaling by MDA5
Wu B, Peisley A, Richards C, Yao H, Zeng Z, Lin C, Chu F, Walz T & Hur S. Cell, (2013) 152, 276-89, PMID: 23273991. Highlighted in Nat. Rev. Immunol. (2013), 13:153. PDF
Multi-level regulation of cellular recognition of viral dsRNA
Peisley A & Hur S. Cell Mol. Life Sci. (2013), 70, 1949-63, PMID:22960755 (Review). PDF
2011 - 2012
MDA5 detects the double-stranded RNA replicative form in picornavirus-infected cells
Feng Q, Hato SV, Langereis MA, Zoll J, Virgen-Slane R, Peisley A, Hur S, Semler BL, van Rij R & van Kuppeveld FJM. Cell Reports, (2012), 2, 1187-96, PMID:23142662. PDF
Kinetic Mechanism for Viral dsRNA Length Discrimination by MDA5 filament
Peisley A, Jo MH, Lin C, Wu B, Orme-Johnson M, Walz T, Hohng S & Hur S. Proc. Natl. Acad. Sci. U.S.A. (2012), 109, E3340-9, PMID:23129641. PDF
Cooperative Assembly and Dynamic Disassembly of MDA5 Filaments for Viral dsRNA Recognition
Peisley A, Lin C, Wu B, Orme-Johnson M, Liu M, Walz T & Hur S. Proc. Natl. Acad. Sci. U.S.A. (2011), 108, 21010-5. PDF