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- 01GJF7PNZSXX5JTJNKGV27FJAB classification A1.
- 01GJF7PNZSXX5JTJNKGV27FJAB date "2022".
- 01GJF7PNZSXX5JTJNKGV27FJAB language "eng".
- 01GJF7PNZSXX5JTJNKGV27FJAB type journalArticle.
- 01GJF7PNZSXX5JTJNKGV27FJAB hasPart 01GKKBY0FJ5C2NQG12Z8EK8JTQ.pdf.
- 01GJF7PNZSXX5JTJNKGV27FJAB subject "Biology and Life Sciences".
- 01GJF7PNZSXX5JTJNKGV27FJAB subject "Medicine and Health Sciences".
- 01GJF7PNZSXX5JTJNKGV27FJAB doi "10.1126/sciimmunol.abq4531".
- 01GJF7PNZSXX5JTJNKGV27FJAB issn "2470-9468".
- 01GJF7PNZSXX5JTJNKGV27FJAB issue "77".
- 01GJF7PNZSXX5JTJNKGV27FJAB volume "7".
- 01GJF7PNZSXX5JTJNKGV27FJAB abstract "Herpes simplex virus 1 (HSV-1) infects several billion people worldwide and can cause life-threatening herpes simplex encephalitis (HSE) in some patients. Monogenic defects in components of the type I interferon system have been identified in patients with HSE, emphasizing the role of inborn errors of immunity underlying HSE pathogenesis. Here, we identify compound heterozygous loss-of-function mutations in the gene GTF3A encoding for transcription factor IIIA (TFIIIA), a component of the RNA polymerase III complex, in a patient with common variable immunodeficiency and HSE. Patient fibroblasts and GTF3A gene–edited cells displayed impaired HSV-1–induced innate immune responses and enhanced HSV-1 replication. Chromatin immunoprecipitation sequencing analysis identified the 5S ribosomal RNA pseudogene 141 (RNA5SP141), an endogenous ligand of the RNA sensor RIG-I, as a transcriptional target of TFIIIA. GTF3A mutant cells exhibited diminished RNA5SP141 expression and abrogated RIG-I activation upon HSV-1 infection. Our work unveils a crucial role for TFIIIA in transcriptional regulation of a cellular RIG-I agonist and shows that GTF3A genetic defects lead to impaired cell-intrinsic anti–HSV-1 responses and can predispose to HSE.".
- 01GJF7PNZSXX5JTJNKGV27FJAB author 0109AD1C-F0EE-11E1-A9DE-61C894A0A6B4.
- 01GJF7PNZSXX5JTJNKGV27FJAB author 0242DD34-F0EE-11E1-A9DE-61C894A0A6B4.
- 01GJF7PNZSXX5JTJNKGV27FJAB author 118A0826-F0EE-11E1-A9DE-61C894A0A6B4.
- 01GJF7PNZSXX5JTJNKGV27FJAB author 13CBBADE-2177-11E3-825E-776F10BDE39D.
- 01GJF7PNZSXX5JTJNKGV27FJAB author 4054C2A2-2240-11E3-BD2F-D1D610BDE39D.
- 01GJF7PNZSXX5JTJNKGV27FJAB author B31CACEE-B220-11E8-96F8-E0445707D3EF.
- 01GJF7PNZSXX5JTJNKGV27FJAB author F43C694E-F0ED-11E1-A9DE-61C894A0A6B4.
- 01GJF7PNZSXX5JTJNKGV27FJAB author F49E9858-F0ED-11E1-A9DE-61C894A0A6B4.
- 01GJF7PNZSXX5JTJNKGV27FJAB author F4A35410-F0ED-11E1-A9DE-61C894A0A6B4.
- 01GJF7PNZSXX5JTJNKGV27FJAB author F50FFF8E-F0ED-11E1-A9DE-61C894A0A6B4.
- 01GJF7PNZSXX5JTJNKGV27FJAB author F552F726-F0ED-11E1-A9DE-61C894A0A6B4.
- 01GJF7PNZSXX5JTJNKGV27FJAB author F5D94AA6-F0ED-11E1-A9DE-61C894A0A6B4.
- 01GJF7PNZSXX5JTJNKGV27FJAB author F81EEFFA-F0ED-11E1-A9DE-61C894A0A6B4.
- 01GJF7PNZSXX5JTJNKGV27FJAB author F8B46BC0-F0ED-11E1-A9DE-61C894A0A6B4.
- 01GJF7PNZSXX5JTJNKGV27FJAB author F946B58E-F0ED-11E1-A9DE-61C894A0A6B4.
- 01GJF7PNZSXX5JTJNKGV27FJAB author urn:uuid:052354ba-5583-4acb-a587-cfa693c93320.
- 01GJF7PNZSXX5JTJNKGV27FJAB author urn:uuid:05a2d622-a086-4dbf-97d8-0833bff4c98b.
- 01GJF7PNZSXX5JTJNKGV27FJAB author urn:uuid:2f783898-646a-4eef-b42a-cdba1eb9fe0a.
- 01GJF7PNZSXX5JTJNKGV27FJAB author urn:uuid:7baaa099-f23e-4815-82be-e3f1ba002e8f.
- 01GJF7PNZSXX5JTJNKGV27FJAB author urn:uuid:e0547f02-eb99-4cb0-b571-9583f65f6ca5.
- 01GJF7PNZSXX5JTJNKGV27FJAB author urn:uuid:eab83481-f665-4fa3-a582-ffa3899feb9f.
- 01GJF7PNZSXX5JTJNKGV27FJAB dateCreated "2022-11-22T08:36:34Z".
- 01GJF7PNZSXX5JTJNKGV27FJAB dateModified "2024-12-12T20:47:47Z".
- 01GJF7PNZSXX5JTJNKGV27FJAB name "GTF3A mutations predispose to herpes simplex encephalitis by disrupting biogenesis of the host-derived RIG-I ligand RNA5SP141".
- 01GJF7PNZSXX5JTJNKGV27FJAB pagination urn:uuid:cee3f0fc-02a9-4768-8404-98f393b0bd00.
- 01GJF7PNZSXX5JTJNKGV27FJAB sameAs LU-01GJF7PNZSXX5JTJNKGV27FJAB.
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- 01GJF7PNZSXX5JTJNKGV27FJAB sourceOrganization urn:uuid:4e09122a-024c-4c13-bb9c-7de4d4421dd9.
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- 01GJF7PNZSXX5JTJNKGV27FJAB sourceOrganization urn:uuid:76633413-68e3-4a45-8143-a146322546bf.
- 01GJF7PNZSXX5JTJNKGV27FJAB sourceOrganization urn:uuid:90908eb2-3c3f-4dcb-b670-a4328b23da96.
- 01GJF7PNZSXX5JTJNKGV27FJAB type A1.