The Nuclear Receptor Nor-1 Is Essential for Proliferation of the Semicircular Canals of the Mouse Inner Ear
| Autor: | Quiana Burton, Tiia Ponnio, Orla M. Conneely, Doris K. Wu, Fred A. Pereira |
|---|---|
| Rok vydání: | 2002 |
| Předmět: |
Receptors
Steroid Membranous labyrinth Receptors Cytoplasmic and Nuclear Apoptosis Nerve Tissue Proteins Biology Epithelium Mice Mammalian Genetic Models with Minimal or Complex Phenotypes otorhinolaryngologic diseases medicine Animals Inner ear Ampulla Postural Balance Molecular Biology Transcription factor Mice Knockout Vestibular system Receptors Thyroid Hormone Semicircular canal Gene Expression Regulation Developmental Cell Biology Anatomy Semicircular Canals DNA-Binding Proteins Mice Inbred C57BL medicine.anatomical_structure Nuclear receptor Vestibule sense organs Cell Division |
| Zdroj: | Molecular and Cellular Biology. 22:935-945 |
| ISSN: | 1098-5549 |
| DOI: | 10.1128/mcb.22.3.935-945.2002 |
| Popis: | Nor-1 belongs to the nur subfamily of nuclear receptor transcription factors. The precise role of Nor-1 in mammalian development has not been established. However, recent studies indicate a function for this transcription factor in oncogenesis and apoptosis. To examine the spatiotemporal expression pattern of Nor-1 and the developmental and physiological consequences of Nor-1 ablation, Nor-1-null mice were generated by insertion of the lacZ gene into the Nor-1 genomic locus. Disruption of the Nor-1 gene results in inner ear defects and partial bidirectional circling behavior. During early otic development, Nor-1 is expressed exclusively in the semicircular canal forming fusion plates. After formation of the membranous labyrinth, Nor-1 expression in the vestibule is limited to nonsensory epithelial cells localized at the inner edge of the semicircular canals and to the ampullary and utricular walls. In the absence of Nor-1, the vestibular walls fuse together as normal; however, the endolymphatic fluid space in the semicircular canals is diminished and the roof of the ampulla appears flattened due to defective continual proliferative growth of the semicircular canals. |
| Databáze: | OpenAIRE |
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