... to be spatially restricted and temporally dynamic throughout inner ear development. We evaluated these characteristics using genetic labeling mediated by Fgf8 mcm gene-targeted mice and determined that Fgf8 expression is a specific and early marker of Type-I vestibular hair cell identity. Fgf8 mcm expression...

... alone. Based on these findings, we propose a dual-lock model where secreted Wnts and Vangl2 cooperate to ensure proper HC polarization in the embryonic cochlea ( Fig. 7 ). Fig. 7. Secreted Wnts and Vangl2 coordinate to regulate hair cell orientation. Proposed ‘dual lock’ model where secreted...

... delays the differentiation of mechano-sensory hair cells (HCs). To address whether the role of let-7 in auditory-sensory differentiation is conserved among vertebrates, we manipulated let-7 levels within the chicken auditory organ: the basilar papilla. Using a let-7 sponge construct to sequester let-7...

...Tomoko Tateya; Susumu Sakamoto; Fumiyoshi Ishidate; Tsuyoshi Hirashima; Itaru Imayoshi; Ryoichiro Kageyama ABSTRACT During cochlear development, hair cells (HCs) and supporting cells differentiate in the prosensory domain to form the organ of Corti, but how one row of inner HCs (IHCs) and three...

...Will J. McLean; Dalton T. McLean; Ruth Anne Eatock; Albert S. B. Edge Disorders of hearing and balance are most commonly associated with damage to cochlear and vestibular hair cells or neurons. Although these cells are not capable of spontaneous regeneration, progenitor cells in the hearing...

...Basile Tarchini; Abigail L. D. Tadenev; Nicholas Devanney; Michel Cayouette Sensory perception in the inner ear relies on the hair bundle, the highly polarized brush of movement detectors that crowns hair cells. We previously showed that, in the mouse cochlea, the edge of the forming bundle...

...Joana Neves; Carolina Parada; Mireia Chamizo; Fernando Giráldez Hair cells of the inner ear sensory organs originate from progenitor cells located at specific domains of the otic vesicle: the prosensory patches. Notch signalling is necessary for sensory development and loss of function of the Notch...

... foxj1b . By contrast, the hair cells develop immotile kinocilia that serve as static tethers for otolith crystallization. In ears devoid of all cilia, otoliths can form but they are of irregular shapes and sizes and appear to attach instead to the hair cell apical membranes. Moreover, overproduction...

... of the dynactin complex. Loss of ako function results in a degeneration of photoreceptors and mechanosensory hair cells. Additionally, mutant Müller cells lose apical processes and their perikarya translocate rapidly towards the vitreal surface of the retina. This is accompanied by the accumulation of the apical...

...Norio Yamamoto; Takayuki Okano; Xuefei Ma; Robert S. Adelstein; Matthew W. Kelley The sensory epithelium of the mammalian cochlea comprises mechanosensory hair cells that are arranged into four ordered rows extending along the length of the cochlear spiral. The factors that regulate the alignment...

...), are arranged in adjacent rows that form a boundary between a single row of inner hair cells and three rows of outer hair cells (OHCs). PCs are required for auditory function, as mice lacking PCs owing to a mutation in Fgfr3 are deaf. Here, using in vitro and in vivo techniques, we demonstrate that an Fgf8...

...Nicolas Daudet; Linda Ariza-McNaughton; Julian Lewis Notch signalling is well-known to mediate lateral inhibition in inner ear sensory patches, so as to generate a balanced mixture of sensory hair cells and supporting cells. Recently, however, we have found that ectopic Notch activity at an early...

...Louisa S. Tang; Heather M. Alger; Fred A. Pereira The orphan nuclear receptor COUP-TFI (Nr2f1) regulates many aspects of mammalian development, but little is known about its role in cochlear hair cell and Deiter's support cell development. The COUP-TFI knockout( COUP-TFI -/- ) has a significant...

... inner ear is not reflected in its early development; the lamprey otic vesicle is highly asymmetric about the anteroposterior axis, both morphologically and molecularly, and bears a striking resemblance to the zebrafish otic vesicle. The single sensory macula originates as two foci of hair cells...

...Johanna Mantela; Zhe Jiang; Jukka Ylikoski; Bernd Fritzsch; Eldad Zacksenhaus; Ulla Pirvola Precursors of cochlear and vestibular hair cells of the inner ear exit the cell cycle at midgestation. Hair cells are mitotically quiescent during late-embryonic differentiation stages and postnatally. We...

... a duplication of pcdh15 , one gene retained an essential function in the ear and the other in the eye. * Author for correspondence (e-mail: nicolson@ohsu.edu ) 23 11 2004 ©2005. 2005 Protocadherin 15 Hair cell Photoreceptor Zebrafish Deafness Blindness Outer segment Stereocilia...

... in the hindbrain. The otic vesicle in val/val mutants is smaller than normal, yet produces nearly twice the normal number of hair cells, and some hair cells are produced ectopically between the anterior and posterior maculae. Anterior markers pax5 and nkx5.1 are expressed in expanded domains that include...

...Ping Chen; Jane E. Johnson; Huda Y. Zoghbi; Neil Segil During embryonic development of the inner ear, the sensory primordium that gives rise to the organ of Corti from within the cochlear epithelium is patterned into a stereotyped array of inner and outer sensory hair cells separated from each...

... at the posterior focus eliminated the posterior canal. Canal loss was prevented by co-implantation of BMP4 cell beads next to noggin beads. An antibody to the chick hair cell antigen (HCA) was used to examine sensory cell distribution, which was abnormal only in the affected tissues of noggin-exposed inner ears...

...Bruce B. Riley; Ming-Yung Chiang; Lisa Farmer and Rebecca Heck Recent studies of inner ear development suggest that hair cells and support cells arise within a common equivalence group by cell-cell interactions mediated by Delta and Notch proteins. We have extended these studies by analyzing...