A role of the double-stranded RNA-binding protein PACT in mouse ear development and hearing

Theresa M. Rowe, Mark Rizzi, Keiko Hirose, Gregory A. Peters, Ganes C. Sen

Research output: Contribution to journalArticlepeer-review

57 Scopus citations

Abstract

To determine the physiological functions of the mammalian double-stranded RNA-binding protein PACT, the single-copy mouse Pact gene was disrupted and expression of the protein was completely ablated. The most notable phenotypes of the Pact-/- mouse were reduced size and severe microtia. As a result of the congenital abnormality of both outer and middle ears, these mice were hearing impaired. In situ hybridization revealed that PACT mRNA was expressed in specific regions of all three parts of the ear in adult and embryonic wild-type mice. Our study demonstrated an essential role of PACT in mammalian ear development and produced the first animal model for studying human microtia.

Original languageEnglish
Pages (from-to)5823-5828
Number of pages6
JournalProceedings of the National Academy of Sciences of the United States of America
Volume103
Issue number15
DOIs
StatePublished - Apr 11 2006

Keywords

  • Antiviral
  • Gene disruption
  • Innate immunity
  • Microtia
  • PKR

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