Modeling of TREX1-Dependent Autoimmune Disease using Human Stem Cells Highlights L1 Accumulation as a Source of Neuroinflammation

Cell Stem Cell

Volumn 21, Issue 3, 2017, Pages 319-331.e8

  Thomas, Charles A ^a,b,c   Tejwani, Leon ^a,b,c,d   Trujillo, Cleber A ^a,b,c   Negraes, Priscilla D ^a,b,c   Herai, Roberto H ^a,b,c,e   Mesci, Pinar ^a,b,c   Macia, Angela ^a,b,c   Crow, Yanick J ^f,g   Muotri, Alysson R ^a,b,c  

^a RADY CHILDREN'S HOSPITAL   (United States)

^b UNIVERSITY OF CALIFORNIA   (United States)

^c San Diego   (United States)

^d YALE UNIVERSITY   (United States)

^e PONTIFICAL CATHOLIC UNIVERSITY OF PARANÁ   (Brazil)

^f INSERM   (France)

^g UNIVERSITY OF MANCHESTER   (United Kingdom)

Author keywords

Aicardi Gouti res syndrome; disease modeling; LINE 1; neuroinflammation; TREX1; type I IFN

Indexed keywords

EXTRACHROMOSOMAL DNA; INTERFERON; THREE PRIME REPAIR EXONUCLEASE 1; UNCLASSIFIED DRUG; VIRUS ENZYME; CELL EXTRACT; DNA; EXODEOXYRIBONUCLEASE; PHOSPHOPROTEIN;

AICARDI GOUTIERES SYNDROME; APOPTOSIS; ARTICLE; ASTROCYTE; AUTOIMMUNE DISEASE; CELLULAR DISTRIBUTION; CONTROLLED STUDY; CYTOPLASM; ENZYME ACTIVITY; HUMAN; HUMAN CELL; LONG INTERSPERSED REPEAT; NERVOUS SYSTEM INFLAMMATION; NEUROTOXICITY; NONSENSE MEDIATED MRNA DECAY; PHENOTYPE; PLURIPOTENT STEM CELL; PRIORITY JOURNAL; RETROPOSON; WHOLE EXOME SEQUENCING; CHILD; CYTOSOL; DEFICIENCY; DRUG EFFECT; ENZYMOLOGY; GENETICS; INFANT; INFLAMMATION; MALE; METABOLISM; MICROCEPHALY; NERVE CELL; NERVOUS SYSTEM; NEURAL STEM CELL; NEWBORN; NUCLEOTIDE SEQUENCE; ORGANOID; PATHOLOGY; STEM CELL; UPREGULATION;

ASTROCYTES; AUTOIMMUNE DISEASES; BASE SEQUENCE; CELL EXTRACTS; CHILD; CYTOSOL; DNA; EXODEOXYRIBONUCLEASES; HUMANS; INFANT; INFANT, NEWBORN; INFLAMMATION; INTERFERONS; LONG INTERSPERSED NUCLEOTIDE ELEMENTS; MALE; MICROCEPHALY; NERVOUS SYSTEM; NEURAL STEM CELLS; NEURONS; ORGANOIDS; PHENOTYPE; PHOSPHOPROTEINS; STEM CELLS; UP-REGULATION;

EID: 85028318361     PISSN: 19345909     EISSN: 18759777     Source Type: Journal    
DOI: 10.1016/j.stem.2017.07.009     Document Type: Article

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