Linked Life Data

10407036

Source:http://linkedlifedata.com/resource/pubmed/id/10407036

Statements in which the resource exists as a subject.
PredicateObject
rdf:type
lifeskim:mentions
pubmed:issue
14
pubmed:dateCreated
1999-8-2
pubmed:abstractText
Targeted mutagenesis in mice demonstrates that the POU-domain gene Brn4/Pou3f4 plays a crucial role in the patterning of the mesenchymal compartment of the inner ear. Brn4 is expressed extensively throughout the condensing mesenchyme of the developing inner ear. Mutant animals displayed behavioral anomalies that resulted from functional deficits in both the auditory and vestibular systems, including vertical head bobbing, changes in gait, and hearing loss. Anatomical analyses of the temporal bone, which is derived in part from the otic mesenchyme, demonstrated several dysplastic features in the mutant animals, including enlargement of the internal auditory meatus. Many phenotypic features of the mutant animals resulted from the reduction or thinning of the bony compartment of the inner ear. Histological analyses demonstrated a hypoplasia of those regions of the cochlea derived from otic mesenchyme, including the spiral limbus, the scala tympani, and strial fibrocytes. Interestingly, we observed a reduction in the coiling of the cochlea, which suggests that Brn-4 plays a role in the epithelial-mesenchymal communication necessary for the cochlear anlage to develop correctly. Finally, the stapes demonstrated several malformations, including changes in the size and morphology of its footplate. Because the stapes anlage does not express the Brn4 gene, stapes malformations suggest that the Brn4 gene also plays a role in mesenchymal-mesenchymal signaling. On the basis of these data, we suggest that Brn-4 enhances the survival of mesodermal cells during the mesenchymal remodeling that forms the mature bony labyrinth and regulates inductive signaling mechanisms in the otic mesenchyme.
pubmed:grant
pubmed:language
eng
pubmed:journal
pubmed:citationSubset
IM
pubmed:chemical
pubmed:status
MEDLINE
pubmed:month
Jul
pubmed:issn
1529-2401
pubmed:author
pubmed:issnType
Electronic
pubmed:day
15
pubmed:volume
19
pubmed:owner
NLM
pubmed:authorsComplete
Y
pubmed:pagination
5980-9
pubmed:dateRevised
2007-11-15
pubmed:meshHeading
pubmed-meshheading:10407036-Acoustic Stimulation, pubmed-meshheading:10407036-Animals, pubmed-meshheading:10407036-Auditory Perception, pubmed-meshheading:10407036-Cochlea, pubmed-meshheading:10407036-DNA-Binding Proteins, pubmed-meshheading:10407036-Ear, Inner, pubmed-meshheading:10407036-Female, pubmed-meshheading:10407036-Gait, pubmed-meshheading:10407036-Gene Expression Regulation, Developmental, pubmed-meshheading:10407036-Hearing Disorders, pubmed-meshheading:10407036-Male, pubmed-meshheading:10407036-Mice, pubmed-meshheading:10407036-Mice, Inbred C57BL, pubmed-meshheading:10407036-Mice, Inbred Strains, pubmed-meshheading:10407036-Mice, Knockout, pubmed-meshheading:10407036-Mice, Transgenic, pubmed-meshheading:10407036-Motor Activity, pubmed-meshheading:10407036-Mutagenesis, Site-Directed, pubmed-meshheading:10407036-Nerve Tissue Proteins, pubmed-meshheading:10407036-POU Domain Factors, pubmed-meshheading:10407036-Recombinant Fusion Proteins, pubmed-meshheading:10407036-Reflex, pubmed-meshheading:10407036-Restriction Mapping, pubmed-meshheading:10407036-Semicircular Canals, pubmed-meshheading:10407036-Stapes, pubmed-meshheading:10407036-Transcription Factors, pubmed-meshheading:10407036-Vestibule, Labyrinth, pubmed-meshheading:10407036-beta-Galactosidase
pubmed:year
1999
pubmed:articleTitle
Targeted mutagenesis of the POU-domain gene Brn4/Pou3f4 causes developmental defects in the inner ear.
pubmed:affiliation
Department of Neuroscience, Head and Neck Surgery, University of Pennsylvania, Philadelphia, Pennsylvania 19104-6074, USA.
pubmed:publicationType
Journal Article, Research Support, U.S. Gov't, P.H.S., Research Support, Non-U.S. Gov't