Hop is an unusual homeobox gene that modulates cardiac development

Fabian Chen, Hyun Kook, Rita Milewski, Aaron D. Gitler, Min Min Lu, Jun Li, Ronniel Nazarian, Robert Schnepp, Kuang-Yu Jen, Christine Biben, Greg Runke, Joel P. Mackay, Jiri Novotny, Robert J. Schwartz, Richard P. Harvey, Mary C. Mullins, Jonathan A. Epstein

Research output: Contribution to journalArticlepeer-review

222 Scopus citations


Hop is a small, divergent homeodomain protein that lacks certain conserved residues required for DNA binding. Hop gene expression initiates early in cardiogenesis and continues in cardiomyocytes throughout embryonic and postnatal development. Genetic and biochemical data indicate that Hop functions directly downstream of Nkx2-5. Inactivation of Hop in mice by homologous recombination results in a partially penetrant embryonic lethal phenotype with severe developmental cardiac defects involving the myocardium. Inhibition of Hop activity in zebrafish embryos likewise disrupts cardiac development and results in severely impaired cardiac function. Hop physically interacts with serum response factor (SRF) and inhibits activation of SRF-dependent transcription by inhibiting SRF binding to DNA. Hop encodes an unusual homeodomain protein that modulates SRF-dependent cardiac-specific gene expression and cardiac development.

Original languageEnglish (US)
Pages (from-to)713-723
Number of pages11
Issue number6
StatePublished - Sep 20 2002
Externally publishedYes

ASJC Scopus subject areas

  • Biochemistry, Genetics and Molecular Biology(all)


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