Efficient attenuation of Friedreich's ataxia (FRDA) cardiomyopathy by modulation of iron homeostasis-human induced pluripotent stem cell (hiPSC) as a drug screening platform for FRDA

International Journal of Cardiology

Volumn 203, Issue , 2016, Pages 964-971

  Lee, Yee Ki ^a   Lau, Yee Man ^a   Ng, Kwong Man ^a   Lai, Wing Hon ^a   Ho, Shu Leong ^b   Tse, Hung Fat ^a   Siu, Chung Wah ^a   Ho, Philip Wing Lok ^b  

^a UNIVERSITY OF HONG KONG   (Hong Kong)

^b UNIVERSITY OF HONG KONG   (Hong Kong)

Author keywords

Cardiomyopathy; Deferiprone; Friedreich's ataxia; Idebenone; Induced pluripotent stem cells

Indexed keywords

CALCIUM; CELL PROTEIN; CYTOCHROME C OXIDASE; DEFERIPRONE; FERRITIN; FERROUS SULFATE; FRATAXIN; IDEBENONE; IRON; IRON RESPONSE PROTEIN 1; REACTIVE OXYGEN METABOLITE; SARCOPLASMIC RETICULUM CALCIUM TRANSPORTING ADENOSINE TRIPHOSPHATASE; SUCCINATE DEHYDROGENASE; TRANSFERRIN RECEPTOR; UNCLASSIFIED DRUG; ANTIOXIDANT; IRON BINDING PROTEIN; IRON CHELATING AGENT; PYRIDONE DERIVATIVE; RNA; UBIQUINONE;

ARTICLE; CALCIUM MOBILIZATION; CARDIOMYOPATHY; CELL LEVEL; CELL STRESS; COMPARATIVE EFFECTIVENESS; CONTROLLED STUDY; DOSE RESPONSE; DRUG EFFECT; DRUG MECHANISM; DRUG SCREENING; EXCITATION CONTRACTION COUPLING; FRIEDREICH ATAXIA; FXN GENE; GENE; GENE CONTROL; GENE EXPRESSION REGULATION; HEART MUSCLE CELL; HOMEOSTASIS; HUMAN; HUMAN CELL; IRON CHELATION; IRON HOMEOSTASIS; IRON TRANSPORT; MITOCHONDRIAL STRESS; MRSC 3 4 GENE; PLURIPOTENT STEM CELL; PRIORITY JOURNAL; PROTEIN EXPRESSION; RESPIRATORY CHAIN; SERCA GENE; TSFR GENE; ANALOGS AND DERIVATIVES; BIOSYNTHESIS; CARDIAC MUSCLE CELL; GENETICS; INDUCED PLURIPOTENT STEM CELL; METABOLISM; OXIDATIVE STRESS; PATHOLOGY; PRECLINICAL STUDY; PROCEDURES; REVERSE TRANSCRIPTION POLYMERASE CHAIN REACTION;

ANTIOXIDANTS; DRUG EVALUATION, PRECLINICAL; FRIEDREICH ATAXIA; GENE EXPRESSION REGULATION; HOMEOSTASIS; HUMANS; INDUCED PLURIPOTENT STEM CELLS; IRON; IRON CHELATING AGENTS; IRON-BINDING PROTEINS; MYOCYTES, CARDIAC; OXIDATIVE STRESS; PYRIDONES; REVERSE TRANSCRIPTASE POLYMERASE CHAIN REACTION; RNA; UBIQUINONE;

EID: 84952837487     PISSN: 01675273     EISSN: 18741754     Source Type: Journal    
DOI: 10.1016/j.ijcard.2015.11.101     Document Type: Article

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