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Publications

2024

PACT prevents aberrant activation of PKR by endogenous dsRNA without sequestration

Ahmad S., Zou T., Zhao L., Wang X., Hwang J., Davydenko A., Buchumenski I., Zhuang P., Fishbein AR., Capcha-Rodriguez D., Orgel A., Levanon EY., Myong S., Chou J., Meyerson M., Hur S. 2024. bioRxiv Preprint. PDF

Mechanism for controlled assembly of transcriptional condensates by Aire

Huoh Y-S., Zhang Q., Torner R., Baca S., Arthanari H. & Hur S. (2024). Nature Immunology. PDF

2023

FOXP3 recognizes microsatellites and bridges DNA through multimerization

Zhang W., Leng F., Wang X., Ramirez R., Park J., Benoist C., Hur S. (2023). Nature 624, 433-441. 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

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