Directly converted patient-specific induced neurons mirror the neuropathology of FUS with disrupted nuclear localization in amyotrophic lateral sclerosis

Molecular Neurodegeneration

Volumn 11, Issue 1, 2016, Pages

  Lim, Su Min ^a,b   Choi, Won Jun ^c   Oh, Ki Wook ^a,b   Xue, Yuanchao ^d   Choi, Ji Young ^b   Kim, Sung Hoon ^b   Nahm, Minyeop ^b   Kim, Young Eun ^e   Lee, Jinhyuk ^f,g   Noh, Min Young ^b   Lee, Seungbok ^h   Hwang, Sejin ^a   Ki, Chang Seok ^e   Fu, Xiang Dong ⁱ   Kim, Seung Hyun ^a,b  

^a Hanyang University   (South Korea)

^b Hanyang University College of Medicine   (South Korea)

^c UAE   (United Arab Emirates)

^d INSTITUTE OF BIOPHYSICS   (China)

^e Sungkyunkwan University School of Medicine   (South Korea)

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

^g University of Science and Technology   (South Korea)

^h Seoul National University   (South Korea)

ⁱ UNIVERSITY OF CALIFORNIA   (United States)

Author keywords

Amyotrophic lateral sclerosis; Fused in sarcoma; Human cell models; Induced neuron; Neuronal cytoplasmic inclusion; Nuclear localization signal; Stress granules

Indexed keywords

FUSED IN SARCOMA PROTEIN; MUTANT PROTEIN; RNA BINDING PROTEIN FUS;

ADULT; AMYOTROPHIC LATERAL SCLEROSIS; ANIMAL CELL; ARTICLE; CARBOXY TERMINAL SEQUENCE; CASE REPORT; CELL GRANULE; CLINICAL FEATURE; CONTROLLED STUDY; CYTOPLASM; FIBROBLAST; FUSED IN SARCOMA GENE; GENE MUTATION; GENETIC TRANSFECTION; HUMAN; HUMAN CELL; HUMAN TISSUE; NERVE CELL DIFFERENTIATION; NEUROPATHOLOGY; NONHUMAN; NUCLEAR LOCALIZATION SIGNAL; OXIDATIVE STRESS; PLURIPOTENT STEM CELL; RAT; RELIABILITY; SEQUENCE ANALYSIS; CELL INCLUSION; CELL NUCLEUS; FEMALE; GENETICS; MALE; METABOLISM; MOTONEURON; MUTATION; PATHOLOGY; PROCEDURES;

ADULT; AMYOTROPHIC LATERAL SCLEROSIS; CELL NUCLEUS; CYTOPLASMIC GRANULES; FEMALE; HUMANS; INCLUSION BODIES; MALE; MOTOR NEURONS; MUTANT PROTEINS; MUTATION; NEUROPATHOLOGY; RNA-BINDING PROTEIN FUS;

EID: 84959328895     PISSN: None     EISSN: 17501326     Source Type: Journal    
DOI: 10.1186/s13024-016-0075-6     Document Type: Article

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