title: ZBTB17 (MIZ1) Is Important for the Cardiac Stress Response and a Novel Candidate Gene for Cardiomyopathy and Heart Failure.
abstract: BACKGROUND
  Mutations in sarcomeric and cytoskeletal proteins are a major cause of hereditary cardiomyopathies, but our knowledge remains incomplete as to how the genetic defects execute their effects.
  
  
  RESULTS
  We used cysteine and glycine-rich protein 3, a known cardiomyopathy gene, in a yeast 2-hybrid screen and identified zinc-finger and BTB domain-containing protein 17 (ZBTB17) as a novel interacting partner. ZBTB17 is a transcription factor that contains the peak association signal (rs10927875) at the replicated 1p36 cardiomyopathy locus. ZBTB17 expression protected cardiac myocytes from apoptosis in vitro and in a mouse model with cardiac myocyte-specific deletion of Zbtb17, which develops cardiomyopathy and fibrosis after biomechanical stress. ZBTB17 also regulated cardiac myocyte hypertrophy in vitro and in vivo in a calcineurin-dependent manner.
  
  
  CONCLUSIONS
  We revealed new functions for ZBTB17 in the heart, a transcription factor that may play a role as a novel cardiomyopathy gene.
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authors.lastname: Milting
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mesh_terms: D000818:Animals; D009202:Cardiomyopathies / Q000235:genetics*; D004268:DNA-Binding Proteins; D006321:Heart / Q000502:physiology; D006333:Heart Failure / Q000235:genetics*; D006801:Humans; D051741:Kruppel-Like Transcription Factors / Q000235:genetics / Q000502:physiology; D060588:LIM Domain Proteins / Q000235:genetics / Q000378:metabolism; D051379:Mice; D009124:Muscle Proteins / Q000235:genetics / Q000378:metabolism; D009687:Nuclear Proteins / Q000235:genetics* / Q000502:physiology; D050828:Protein Inhibitors of Activated STAT / Q000235:genetics / Q000502:physiology; D051381:Rats; D013312:Stress, Physiological; D046509:Tissue Culture Techniques; D044767:Ubiquitin-Protein Ligases
chemical_list: D004268:DNA-Binding Proteins; D051741:Kruppel-Like Transcription Factors; D060588:LIM Domain Proteins; D009124:Muscle Proteins; D009687:Nuclear Proteins; D050828:Protein Inhibitors of Activated STAT; C107961:ZBTB17 protein, human; C090114:cysteine and glycine-rich protein 3; C485521:Miz1 protein, mouse; D044767:Ubiquitin-Protein Ligases; C484534:Pias2 protein, rat
keywords: cardiomyopathies; genetics; heart failure; models, animal; mutation