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Publication : The Kruppel-like transcription factor KLF13 is a novel regulator of heart development.

First Author  Lavallée G Year  2006
Journal  EMBO J Volume  25
Issue  21 Pages  5201-13
PubMed ID  17053787 Mgi Jnum  J:115627
Mgi Id  MGI:3692007 Doi  10.1038/sj.emboj.7601379
Citation  Lavallee G, et al. (2006) The Kruppel-like transcription factor KLF13 is a novel regulator of heart development. EMBO J 25(21):5201-13
abstractText  In humans, congenital heart defects occur in 1-2% of live birth, but the molecular mechanisms and causative genes remain unidentified in the majority of cases. We have uncovered a novel transcription pathway important for heart morphogenesis. We report that KLF13, a member of the Kruppel-like family of zinc-finger proteins, is expressed predominantly in the heart, binds evolutionarily conserved regulatory elements on cardiac promoters and activates cardiac transcription. KLF13 is conserved across species and knockdown of KLF13 in Xenopus embryos leads to atrial septal defects and hypotrabeculation similar to those observed in humans or mice with hypomorphic GATA-4 alleles. Physical and functional interaction with GATA-4, a dosage-sensitive cardiac regulator, provides a mechanistic explanation for KLF13 action in the heart. The data demonstrate that KLF13 is an important component of the transcription network required for heart development and suggest that KLF13 is a GATA-4 modifier; by analogy to other GATA-4 collaborators, mutations in KLF13 may be causative for congenital human heart disease.
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