Poly(GR) in C9ORF72-Related ALS/FTD Compromises Mitochondrial Function and Increases Oxidative Stress and DNA Damage in iPSC-Derived Motor Neurons

Neuron

Volumn 92, Issue 2, 2016, Pages 383-391

  Lopez Gonzalez, Rodrigo ^a   Lu, Yubing ^a   Gendron, Tania F ^b   Karydas, Anna ^c   Tran, Helene ^a   Yang, Dejun ^a   Petrucelli, Leonard ^b   Miller, Bruce L ^c   Almeida, Sandra ^a   Gao, Fen Biao ^a  

^a UNIVERSITY OF MASSACHUSETTS MEDICAL SCHOOL   (United States)

^b MAYO CLINIC   (United States)

^c UNIVERSITY OF CALIFORNIA   (United States)

Author keywords

ALS; C9ORF72; DNA damage; DPR; FTD; iPSC; mitochondria; oxidative stress; RAN translation; repeats

Indexed keywords

PEPTIDES AND PROTEINS; PROTEIN C9ORF72; PROTEIN POLY(GR)80; RIBOSOME PROTEIN; UNCLASSIFIED DRUG; ARGININE; C9ORF72 PROTEIN, HUMAN; DIPEPTIDE; GLYCINE; PROTEIN; PROTEIN P53; REPETITIVE DNA; TP53 PROTEIN, HUMAN;

AGING; AMYOTROPHIC LATERAL SCLEROSIS; ARTICLE; CASE REPORT; CELL DIFFERENTIATION; CONTROLLED STUDY; CYTOTOXICITY; DISORDERS OF MITOCHONDRIAL FUNCTIONS; DNA DAMAGE; FRONTOTEMPORAL DEMENTIA; HUMAN; HUMAN CELL; INDUCED PLURIPOTENT STEM CELL; MOLECULAR PATHOLOGY; MOTONEURON; NONHUMAN; OXIDATIVE STRESS; PRIORITY JOURNAL; PROTEIN BINDING; PROTEIN EXPRESSION; PROTEIN PROTEIN INTERACTION; CELL LINE; GENETICS; METABOLISM; MITOCHONDRION; WESTERN BLOTTING;

AMYOTROPHIC LATERAL SCLEROSIS; ARGININE; BLOTTING, WESTERN; CELL LINE; DIPEPTIDES; DNA DAMAGE; DNA REPEAT EXPANSION; FRONTOTEMPORAL DEMENTIA; GLYCINE; HUMANS; INDUCED PLURIPOTENT STEM CELLS; MITOCHONDRIA; MOTOR NEURONS; OXIDATIVE STRESS; PROTEINS; TUMOR SUPPRESSOR PROTEIN P53;

EID: 84992058801     PISSN: 08966273     EISSN: 10974199     Source Type: Journal    
DOI: 10.1016/j.neuron.2016.09.015     Document Type: Article

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