Autoinflammatory mutation in NLRC4 reveals a leucine-rich repeat (LRR)–LRR oligomerization interface

Journal of Allergy and Clinical Immunology

Volumn 142, Issue 6, 2018, Pages 1956-1967.e6

  Moghaddas, Fiona ^a,b   Zeng, Ping ^c   Zhang, Yuxia ^d   Schützle, Heike ^e   Brenner, Sebastian ^e   Hofmann, Sigrun R ^e   Berner, Reinhard ^e   Zhao, Yuanbo ^d,f   Lu, Bingtai ^d   Chen, Xiaoyun ^d   Zhang, Li ^d   Cheng, Suyun ^c   Winkler, Stefan ^e   Lehmberg, Kai ^g   Canna, Scott W ^h   Czabotar, Peter E ^a,b   Wicks, Ian P ^a,b,i   De Nardo, Dominic ^a,b   Hedrich, Christian M ^e,j,k   Zeng, Huasong ^c   Masters, Seth L ^a,b,d   more..

^a WALTER AND ELIZA HALL INSTITUTE OF MEDICAL RESEARCH   (Australia)

^b UNIVERSITY OF MELBOURNE   (Australia)

^c Guangzhou Women and Children's Medical Centre   (China)

^d Institute of Industry Technology   (China)

^e DRESDEN UNIVERSITY OF TECHNOLOGY   (Germany)

^f GUIZHOU MEDICAL UNIVERSITY   (China)

^g UNIVERSITY MEDICAL CENTER HAMBURG EPPENDORF   (Germany)

^h CHILDREN S HOSPITAL OF PITTSBURGH   (United States)

ⁱ ROYAL MELBOURNE HOSPITAL   (Australia)

^j UNIVERSITY OF LIVERPOOL   (United Kingdom)

^k ALDER HEY CHILDREN'S NHS FOUNDATION TRUST   (United Kingdom)

more..

Author keywords

Autoinflammatory disease; IL 18; inflammasome; IPAF; macrophage activation syndrome; NLRC4; Nod like receptor; periodic fever syndrome

Indexed keywords

ACUTE PHASE PROTEIN; ALANINE; AMINOTRANSFERASE; ANAKINRA; ASPARTIC ACID; C REACTIVE PROTEIN; CASPASE 8; CASPASE RECRUITMENT DOMAIN SIGNALING PROTEIN; CEFTRIAXONE; COMPLEMENTARY DNA; CORTICOSTEROID; CRYOPYRIN; ECULIZUMAB; FERRITIN; INFLAMMASOME; INTERLEUKIN 18; INTERLEUKIN 1BETA; INTERLEUKIN 1BETA CONVERTING ENZYME; INTERLEUKIN 2 RECEPTOR; LEUCINE RICH REPEAT; LEUCINE RICH REPEAT PROTEIN; NOD LIKE RECEPTOR FAMILY CARDCONTAINING 4 PROTEIN; NUCLEOTIDE BINDING OLIGOMERIZATION DOMAIN LIKE RECEPTOR; PROTEIN; SERINE; TOCILIZUMAB; TRIACYLGLYCEROL; UNCLASSIFIED DRUG; CALCIUM BINDING PROTEIN; LEUCINE; NLRC4 PROTEIN, HUMAN;

ABNORMAL RESPIRATORY SOUND; ANTIMICROBIAL THERAPY; APOPTOSIS; ARTICLE; AUTOINFLAMMATORY DISEASE; BONE MARROW BIOPSY; BRONCHOPNEUMONIA; CELL DEATH; CELL STIMULATION; CEREBROSPINAL FLUID; CLINICAL ARTICLE; COMPLEX FORMATION; CRISPR CAS SYSTEM; CRISPR-CAS9 SYSTEM; CYTOKINE RELEASE; CYTOKINE RESPONSE; DIARRHEA; DRUG DOSE INCREASE; DRUG MEGADOSE; DYSPNEA; ECCHYMOSIS; ENZYME LINKED IMMUNOSORBENT ASSAY; FEVER; FLOW CYTOMETRY; GENE MUTATION; GENETIC ANALYSIS; HEK293T CELL LINE; HEPATOMEGALY; HEPATOSPLENOMEGALY; HUMAN; IN VITRO STUDY; INNATE IMMUNITY; KIDNEY FAILURE; KIDNEY INJURY; MACROPHAGE ACTIVATION SYNDROME; MACULOPAPULAR RASH; MUCOSAL BLEEDING; NEWBORN; OLIGOMERIZATION; PANCYTOPENIA; PERITONEAL DIALYSIS; PETECHIA; PRIORITY JOURNAL; PYROPTOSIS; RASH; SINGLE DRUG DOSE; SITE DIRECTED MUTAGENESIS; THORAX RADIOGRAPHY; THP-1 CELL LINE; THROMBOCYTOPENIA; TRIACYLGLYCEROL LEVEL; TYPE III SECRETION SYSTEM; URTICARIA; WESTERN BLOTTING; WHEEZING; WHOLE EXOME SEQUENCING; CASE REPORT; CHEMISTRY; FEMALE; GENETICS; HEK293 CELL LINE; IMMUNOLOGY; INFANT; MACROPHAGE ACTIVATION; MALE; PROTEIN DOMAIN; SYNDROME;

CALCIUM-BINDING PROTEINS; CARD SIGNALING ADAPTOR PROTEINS; FEMALE; HEK293 CELLS; HUMANS; INFANT; INFANT, NEWBORN; INFLAMMASOMES; LEUCINE; MACROPHAGE ACTIVATION; MALE; PROTEIN DOMAINS; SYNDROME; THP-1 CELLS;

EID: 85047923681     PISSN: 00916749     EISSN: 10976825     Source Type: Journal    
DOI: 10.1016/j.jaci.2018.04.033     Document Type: Article

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