| Literature DB >> 16571658 |
Theresa M Rowe1, Mark Rizzi, Keiko Hirose, Gregory A Peters, Ganes C Sen.
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.Entities:
Mesh：
Substances：
Year: 2006 PMID： 16571658 PMCID： PMC1458657 DOI： 10.1073/pnas.0601287103
Source DB: PubMed Journal: Proc Natl Acad Sci U S A ISSN： 0027-8424 Impact factor: 11.205