About: Ribonuclease L mediates the cell-lethal phenotype of double-stranded RNA editing enzyme ADAR1 deficiency in a human cell line

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About: Ribonuclease L mediates the cell-lethal phenotype of double-stranded RNA editing enzyme ADAR1 deficiency in a human cell line Goto Sponge NotDistinct Permalink

An Entity of Type : schema:ScholarlyArticle, within Data Space : covidontheweb.inria.fr associated with source document(s)

Attributes	Values
type	Academic Article research paper schema:ScholarlyArticle
isDefinedBy	Covid-on-the-Web dataset
has title	Ribonuclease L mediates the cell-lethal phenotype of double-stranded RNA editing enzyme ADAR1 deficiency in a human cell line
Creator	Weiss, Susan Li, Yize Banerjee, Shuvojit Dong, Beihua Gaughan, Christina Donovan, Jesse Korennykh, Alexei Rath, Sneha Goldstein, Stephen Silverman, Robert
Source	Medline; PMC
abstract	ADAR1 isoforms are adenosine deaminases that edit and destabilize double-stranded RNA reducing its immunostimulatory activities. Mutation of ADAR1 leads to a severe neurodevelopmental and inflammatory disease of children, Aicardi-Goutiéres syndrome. In mice, Adar1 mutations are embryonic lethal but are rescued by mutation of the Mda5 or Mavs genes, which function in IFN induction. However, the specific IFN regulated proteins responsible for the pathogenic effects of ADAR1 mutation are unknown. We show that the cell-lethal phenotype of ADAR1 deletion in human lung adenocarcinoma A549 cells is rescued by CRISPR/Cas9 mutagenesis of the RNASEL gene or by expression of the RNase L antagonist, murine coronavirus NS2 accessory protein. Our result demonstrate that ablation of RNase L activity promotes survival of ADAR1 deficient cells even in the presence of MDA5 and MAVS, suggesting that the RNase L system is the primary sensor pathway for endogenous dsRNA that leads to cell death. DOI: http://dx.doi.org/10.7554/eLife.25687.001
has issue date	2017-03-31(xsd:dateTime)
bibo:doi	10.7554/elife.25687
bibo:pmid	28362255
has license	cc-by
sha1sum (hex)	02049ffbdb20997055c4def3516af0e6e40dd427
schema:url	https://doi.org/10.7554/elife.25687
resource representing a document's title	Ribonuclease L mediates the cell-lethal phenotype of double-stranded RNA editing enzyme ADAR1 deficiency in a human cell line
has PubMed Central identifier	PMC5404912
has PubMed identifier	28362255
schema:publication	eLife
resource representing a document's body	covid:02049ffbdb20997055c4def3516af0e6e40dd427#body_text
is schema:about of	named entity 'survival' named entity 'expression' named entity 'MAVS' named entity 'However' named entity 'proteins' named entity 'neurodevelopmental' named entity 'embryonic' named entity 'deletion' named entity 'mutagenesis' covid:arg/02049ffbdb20997055c4def3516af0e6e40dd427 named entity 'regulated' named entity 'A549' named entity 'lung adenocarcinoma' named entity 'Mda5' named entity 'ADAR1' named entity 'leads' named entity 'NS2' named entity 'adenosine' named entity 'embryonic lethal' named entity 'RNase' named entity 'phenotype' named entity 'isoforms' named entity 'immunostimulatory' named entity 'cell death' named entity 'ADAR1' named entity 'cell line' named entity 'MAVS' named entity 'exogenous' named entity 'tRNA' named entity 'ADAR1' named entity 'Pestal' named entity 'centrifugation' named entity 'mutation' named entity 'eIF2a' named entity 'gene knockout' named entity 'primer' named entity 'Nonidet P-40' named entity 'ISGs' named entity 'eIF2a' named entity 'antiviral' named entity 'OASs' named entity 'mutagenesis' named entity 'ADAR1' named entity 'Cell survival' named entity 'STR analysis' named entity 'Tris-HCl' named entity 'Mda5' named entity 'phosphate' named entity 'DKO' named entity 'IFN' named entity 'CRISPR' named entity 'MAVS' named entity 'ADAR1' named entity 'total protein' named entity 'incubation' named entity 'antibody' named entity 'phosphodiesterase' named entity 'clone' named entity 'ADAR' named entity 'dsRNA' named entity 'nucleic acid' named entity 'virus infections' named entity 'PDE' named entity 'RNase L' named entity 'rRNA' named entity 'mice' named entity 'RNase L' named entity 'ADAR1' named entity 'AGS' named entity 'ADAR1' named entity 'IFN' named entity 'MAVS'

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