ZBTB17 (MIZ1) Is Important for the Cardiac Stress Response and a Novel Candidate Gene for Cardiomyopathy and Heart Failure

Byambajav Buyandelger, Catherine Mansfield, Sawa Kostin, Onjee Choi, Angharad M Roberts, James S Ware, Francesco Mazzarotto, Francesco Pesce, Rachel Buchan, Rivka Isaacson, Josee Vouffo, Sylvia Gunkel, Gudrun Knöll, Sara J McSweeney, Heming Wei, Andreas Perrot, Conny Pfeiffer, Mohammad Reza Toliat, Kristina Ilieva, Ewelina KrysztofinskaMarina M López-Olañeta, Jesús M Gómez-Salinero, Albrecht Schmidt, Keat-Eng Ng, Niels Teucher, Ju Chen, Martin Teichmann, Martin Eilers, Wilhelm Haverkamp, Vera Regitz-Zagrosek, Gerd Hasenfuss, Thomas Braun, Dudley J Pennell, Ian Gould, Paul J R Barton, Enrique Lara-Pezzi, Sebastian Schafer, Norbert Hübner, Leanne E Felkin, Declan P O'Regan, Enrico Petretto, Thomas Brand, Hendrik Milting, Peter Nürnberg, Michael D Schneider, Sanjay Prasad, Ralph Knöll

Research output: Contribution to journalArticlepeer-review

12 Citations (Scopus)


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.

Methods and Results—We used cysteine and glycine-rich protein 3 (CSRP3), a known cardiomyopathy gene, in a yeast two-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 which may play a role as a novel cardiomyopathy gene.
Original languageEnglish
Pages (from-to)643-652
Number of pages9
JournalCirculation-Cardiovascular Genetics
Issue number5
Early online date14 Jul 2015
Publication statusPublished - Oct 2015


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