GEO DataSets

Analysis of neurons and astrocytes derived from UNC-93B-deficient induced pluripotent stem cells (UNC93B-/- iPS) and from hESCs (H9). UNC-93B-/- neurons are highly susceptible to herpes simplex virus 1 (HSV-1) infection. Results provide insight into role of neurons in anti-HSV-1 immunity in the CNS.

Organism:

Homo sapiens

Type:

Expression profiling by array, count, 2 cell line, 3 cell type sets

Platform:

GPL6884

Series:

GSE40593

13 Samples

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(Submitter supplied) In the course of primary infection with herpes simplex virus 1 (HSV-1), children with inborn errors of the TLR3- and UNC-93B-dependent induction of IFN-α/β and/or IFN-λ immunity are prone to HSV-1 encephalitis (HSE) 1-3. The cells responsible for HSE in these children have yet to be identified. We tested the hypothesis that the pathogenesis of HSE involves non hematopoietic central nervous system (CNS)-resident cells. more...

Organism:

Homo sapiens

Type:

Expression profiling by array

Datasets:

GDS4538 GDS4669

Platform:

GPL6884

18 Samples

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Analysis of hESC (H9)-derived CNS cell types: neural rosettes, neurons, astrocytes and immature oligodendrocytes. Results provide insight into molecular profiles of CNS neuronal populations susceptible to herpes simplex virus 1 (HSV-1) infection.

Organism:

Homo sapiens

Type:

Expression profiling by array, count, 4 cell type sets

Platform:

GPL6884

Series:

GSE40593

11 Samples

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(Submitter supplied) Pseudo-seq was used to measure differences in the extent of pseudouridine (Ψ) modification in rRNA from human cell lines with heterozygous, and homozygous disruptions of SNORA31.

Organism:

Homo sapiens

Type:

Other

Platform:

GPL11154

22 Samples

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(Submitter supplied) We report here two unrelated HSE patients carrying different heterozygous mutations (D50A and G159A) in TBK1, the gene encoding TANK-binding kinase 1, a kinase at the crossroads of multiple IFN-inducing signaling pathways. Both mutant TBK1 alleles are loss-of-function, but through different mechanisms: protein instability (D50A) or a loss of kinase activity (G159A). Both are also associated with an autosomal dominant (AD) trait, but by different mechanisms: haplotype-insufficiency (D50A) or negative dominance (G159A). more...

Organism:

Homo sapiens

Type:

Expression profiling by array

Platform:

GPL10558

58 Samples

Download data: TXT

(Submitter supplied) The objective of this study is to study the Toll-like receptor 3 (TLR3)-dependent gene expression in human fibroblast cells and peripheral blood mononuclear cells (PBMCs)

Organism:

Homo sapiens

Type:

Expression profiling by array

Platform:

GPL10558

44 Samples

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(Submitter supplied) We report here unrelated HSE patients with autosomal recessive (AR) or autosomal dominant (AD) TRIF deficiency. The AR form of the disease is due to a homozygous nonsense mutation, resulting in a complete absence of the TRIF protein. Both the TLR3 and the TRIF-dependent TLR4 signaling pathways are abolished. The AD form of TRIF deficiency is due to a heterozygous missense mutation resulting in a dysfunctional protein. more...

Organism:

Homo sapiens

Type:

Expression profiling by array

Datasets:

GDS4539 GDS4540

Platform:

GPL10558

27 Samples

Download data: TXT

Analysis of PBMCs collected from an HSV1 encephalitis (HSE) patient with TRIF deficiency (homozygous TRIF nonsense mutation) and stimulated with LPS or resiquimod hydrochloride R-848. TRIF is the sole adaptor protein for TLR3. Results provide insight into the role of the TLR3 pathway in HSE.

Organism:

Homo sapiens

Type:

Expression profiling by array, transformed count, 3 agent, 2 disease state, 3 genotype/variation sets

Platform:

GPL10558

Series:

GSE32390

9 Samples

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Analysis of SV40 skin fibroblasts collected from an HSV1 encephalitis (HSE) patient with TRIF deficiency (homozygous TRIF nonsense mutation) and stimulated with IL-1β or poly I:C . TRIF is the sole adaptor protein for TLR3. Results provide insight into the role of the TLR3 pathway in HSE.

Organism:

Homo sapiens

Type:

Expression profiling by array, transformed count, 3 agent, 2 disease state, 4 genotype/variation sets

Platform:

GPL10558

Series:

GSE32390

18 Samples

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(Submitter supplied) We hypothesize that human RIPK3 deficiency does not result in impairment of type I IFN mediated antiviral immunity, contrasting with the situation in deficiencies of the TLR3-IFNAR1 circuit. To test the cellular responses to HSV1 at the wide transcriptome level, bulk RNA sequencing was performed with human primary fibroblasts without or with HSV-1 infection for 24 hours, in cells from healthy controls, RIPK3 HSE patient and other patients with recessive TLR3, IFNAR1 or NEMO deficiency.

Organism:

Homo sapiens

Type:

Expression profiling by high throughput sequencing

Platform:

GPL24676

14 Samples

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(Submitter supplied) Inborn errors of TLR3-dependent IFN-α/β- and -λ-mediated immunity in the central nervous system (CNS) can underlie herpes simplex virus 1 (HSV-1) encephalitis (HSE). The respective contributions of IFN-α/β- and -λ are unknown. We report a child homozygous for a genomic deletion of the entire coding sequence and part of the 3’UTR of the last exon of IFNAR1, who died from HSE at the age of two years. An older cousin died following vaccination against measles, mumps and rubella at 12 months of age, and another 17-year-old cousin homozygous for the same variant has had other viral illnesses. The encoded IFNAR1 protein is expressed on the cell surface but is truncated and cannot interact with the tyrosine kinase TYK2. The patient’s fibroblasts and EBV-B cells did not respond to IFN-α2b or IFN-β, in terms of STAT1, STAT2 and STAT3 phosphorylation, or the induction of IFN-stimulated genes (ISGs). Transcriptome analysis revealed a complete abolition of genome-wide ISG induction in response to IFN-α2b in IFNAR1-deficient fibroblasts from the patient. These fibroblasts were susceptible to viruses, including HSV-1, even in the presence of exogenous IFN-α2b or IFN-β. HSE is therefore a consequence of inherited complete IFNAR1 deficiency. This experiment of Nature indicates that IFN-α/β are essential for anti-HSV-1 immunity in the CNS.

Organism:

Homo sapiens

Type:

Expression profiling by high throughput sequencing

Platform:

GPL18573

60 Samples

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(Submitter supplied) This SuperSeries is composed of the SubSeries listed below.

Organism:

Homo sapiens

Type:

Expression profiling by array

Platforms:

GPL17077 GPL10558

24 Samples

Download data: TXT

(Submitter supplied) Herpes simplex virus type 1 (HSV-1) is a 152 Kb double stranded DNA alpha-herpesvirus, which establishes long life latent infection in sensory neurons. Most of our knowledge regarding HSV-1 latency comes from in vivo studies using small animal models, mainly rodents and rabbits, which are not naturally infected by HSV-1. Furthermore, these animal models do not fully recapitulate the species specific effects of human HSV-1 infection. more...

Organism:

Homo sapiens

Type:

Expression profiling by array

Platform:

GPL10558

6 Samples

Download data: TXT

(Submitter supplied) Herpes simplex virus type 1 (HSV-1) is a 152 Kb double stranded DNA alpha-herpesvirus, which establishes long life latent infection in sensory neurons. Most of our knowledge regarding HSV-1 latency comes from in vivo studies using small animal models, mainly rodents and rabbits, which are not naturally infected by HSV-1. Furthermore, these animal models do not fully recapitulate the species specific effects of human HSV-1 infection. more...

Organism:

Homo sapiens

Type:

Expression profiling by array

Platform:

GPL17077

18 Samples

Download data: TXT