Human SNORA31 variations impair cortical neuron-intrinsic immunity to HSV-1 and underlie herpes simplex encephalitis.

doi:10.1038/s41591-019-0672-3

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Article

Human SNORA31 variations impair cortical neuron-intrinsic immunity to HSV-1 and underlie herpes simplex encephalitis.

Nature medicine - 1 Dec 2019

Lafaille Fabien G, Harschnitz Oliver, Lee Yoon Seung, Zhang Peng, Hasek Mary L, Kerner Gaspard, Itan Yuval, Ewaleifoh Osefame, Rapaport Franck, Carlile Thomas M, Carter-Timofte Madalina E, Paquet Dominik, Dobbs Kerry, Zimmer Bastian, Gao Daxing, Rojas-Duran Maria F, Kwart Dylan, Rattina Vimel, Ciancanelli Michael J, McAlpine Jessica L, Lorenzo Lazaro, Boucherit Soraya, Rozenberg Flore, Halwani Rabih, Henry Benoit, Amenzoui Naima, Alsum Zobaida, Marques Laura, Church Joseph A, Al-Muhsen Saleh, Tardieu Marc, Bousfiha Ahmed Aziz, Paludan Søren R, Mogensen Trine Hyrup, Quintana-Murci Lluis, Tessier-Lavigne Marc, Smith Gregory A, Notarangelo Luigi D, Studer Lorenz, Gilbert Wendy, Abel Laurent, Casanova Jean-Laurent, Zhang Shen-Ying

Abstract excerpt

Herpes simplex virus-1 (HSV-1) encephalitis (HSE) is typically sporadic. Inborn errors of TLR3- and DBR1-mediated central nervous system cell-intrinsic immunity can account for forebrain and brainstem HSE, respectively. We report five unrelated patients with forebrain HSE, each heterozygous for one of four rare variants of SNORA31, encoding a small nucleolar RNA of the H/ACA class that are predicted to direct the...

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Identifiers

PMID: 31806906
DOI: 10.1038/s41591-019-0672-3
PMCID: PMC7376819

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PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 7 Sept 2026. Manifest publication-graph-cohort-2026-09-07.1.

Source receipt: pubmed, retrieved 7 Sept 2026.

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