Impaired intrinsic immunity to HSV-1 in human iPSC-derived TLR3-deficient CNS cells

ABSTRACT: 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. We derived induced pluripotent stem cells (iPSCs) from the dermal fibroblasts of TLR3- and UNC-93B-deficient patients and from controls. These iPSCs were allowed to differentiate into highly purified populations of neural stem cells (NSCs), neurons, astrocytes and oligodendrocytes. The induction of IFN-β and/or IFN-λ1 in response to poly(I:C) stimulation was dependent on TLR3

ORGANISM(S): Homo sapiens

SUBMITTER: Jeffrey Zhao 

PROVIDER: E-GEOD-40593 | biostudies-arrayexpress |

REPOSITORIES: biostudies-arrayexpress