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- W4377018737 abstract "Inborn errors of TLR3-dependent type I IFN immunity in cortical neurons underlie forebrain herpes simplex virus-1 (HSV-1) encephalitis (HSE) due to uncontrolled viral growth and subsequent cell death. We report an otherwise healthy patient with HSE who is compound heterozygous for nonsense (R422*) and frameshift (P493fs9*) RIPK3 variants. Receptor interacting protein kinase 3 (RIPK3) is a ubiquitous cytoplasmic kinase regulating cell death outcomes, including apoptosis and necroptosis. In vitro, the R422* and P493fs9* RIPK3 proteins impair cellular apoptosis and necroptosis upon TLR3, TLR4 or TNFR1 stimulation, and ZBP1/DAI-mediated necroptotic cell death following HSV-1 infection. The patient's fibroblasts display no detectable RIPK3 expression. Following TNFR1 or TLR3 stimulation, the patient's cells do not undergo apoptosis or necroptosis. Following HSV-1 infection, the cells support excessive viral growth despite normal induction of antiviral IFN-β and interferon-stimulated genes (ISGs). This phenotype is, nevertheless, rescued by the application of exogenous type I IFN. The patient's human pluripotent stem cell (hPSC)-derived cortical neurons display impaired cell death and enhanced viral growth following HSV-1 infection, as do isogenic RIPK3- knockout hPSC-derived cortical neurons. Inherited RIPK3 deficiency therefore confers a predisposition to HSE, by impairing the cell death-dependent control of HSV-1 in cortical neurons independently of type I IFN immunity." @default.
- W4377018737 created "2023-05-19" @default.
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- W4377018737 date "2023-05-01" @default.
- W4377018737 modified "2023-10-02" @default.
- W4377018737 title "Encephalitis and poor neuronal death-mediated control of herpes simplex virus in human inherited RIPK3 deficiency" @default.
- W4377018737 doi "https://doi.org/10.1016/j.clim.2023.109348" @default.
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