A novel human model of the neurodegenerative disease GM1 gangliosidosis using induced pluripotent stem cells demonstrates inflammasome activation

Journal of Pathology

Volumn 237, Issue 1, 2015, Pages 98-110

  Son, Mi Young ^a,b   Kwak, Jae Eun ^a   Seol, Binna ^a   Lee, Da Yong ^a   Jeon, Hyejin ^a   Cho, Yee Sook ^a,b  

^a Korea Research Institute of Bioscience and Biotechnology (KRIBB)   (South Korea)

^b University of Science and Technology (UST)   (South Korea)

Author keywords

GM1 gangliosidosis; human disease model; induced pluripotent stem cells; inflammasome; neural progenitor cells

Indexed keywords

BETA GALACTOSIDASE; BETA GALACTOSIDASE 1; BETA N ACETYLHEXOSAMINIDASE A; BETA N ACETYLHEXOSAMINIDASE ALPHA CHAIN; BETA N ACETYLHEXOSAMINIDASE BETA CHAIN; COMPLEMENTARY DNA; GANGLIOSIDE GM1; GANGLIOSIDE GM2 ACTIVATOR PROTEIN; INFLAMMASOME; INTERLEUKIN 1 RECEPTOR ACCESSORY PROTEIN; INTERLEUKIN 1 RECEPTOR TYPE I; INTERLEUKIN 1BETA; MESSENGER RNA; SPHINGOLIPID; UNCLASSIFIED DRUG; BIOLOGICAL MARKER; IMMUNOLOGIC FACTOR; INTERLEUKIN 1 RECEPTOR BLOCKING AGENT;

ANIMAL EXPERIMENT; ANIMAL TISSUE; ARTICLE; CELL DIFFERENTIATION; CELL PROLIFERATION; CELL STRUCTURE; CONTROLLED STUDY; DISEASE ASSOCIATION; DISEASE MODEL; DNA SEQUENCE; DOWN REGULATION; EMBRYONIC STEM CELL; ENZYME ACTIVATION; ENZYME ACTIVITY; ENZYME INHIBITION; GENE MUTATION; GM1 GANGLIOSIDOSIS; HUMAN; HUMAN CELL; IN VITRO STUDY; IN VIVO STUDY; LIPID ANALYSIS; MOUSE; NERVOUS SYSTEM INFLAMMATION; NEURAL STEM CELL; NEUROPATHOLOGY; NONHUMAN; NUCLEAR REPROGRAMMING; PATHOGENESIS; PHENOTYPE; PLURIPOTENT STEM CELL; PRIORITY JOURNAL; PROTEIN DEGRADATION; PROTEIN EXPRESSION; SHORT TANDEM REPEAT; SIGNAL TRANSDUCTION; TERATOMA; TUMOR VOLUME; UPREGULATION; ANIMAL; ANTAGONISTS AND INHIBITORS; C57BL MOUSE; CELL LINE; CELL SHAPE; DRUG EFFECTS; GENOTYPE; IMMUNOLOGY; LYSOSOME; METABOLISM; PATHOLOGY; TIME; TRANSPLANTATION;

ANIMALS; BETA-GALACTOSIDASE; BIOLOGICAL MARKERS; CELL LINE; CELL SHAPE; CELLULAR REPROGRAMMING; GANGLIOSIDOSIS, GM1; GENOTYPE; HUMANS; IMMUNOLOGIC FACTORS; INDUCED PLURIPOTENT STEM CELLS; INFLAMMASOMES; INTERLEUKIN 1 RECEPTOR ANTAGONIST PROTEIN; LYSOSOMES; MICE, INBRED C57BL; NEURAL STEM CELLS; PHENOTYPE; SIGNAL TRANSDUCTION; TIME FACTORS;

EID: 84938978012     PISSN: 00223417     EISSN: 10969896     Source Type: Journal    
DOI: 10.1002/path.4551     Document Type: Article

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