scholarly journals Celsr1 coordinates the planar polarity of vestibular hair cells during inner ear development

2017 ◽  
Vol 423 (2) ◽  
pp. 126-137 ◽  
Author(s):  
Jeremy S. Duncan ◽  
Michelle L. Stoller ◽  
Andrew F. Francl ◽  
Fadel Tissir ◽  
Danelle Devenport ◽  
...  
Keyword(s):  
Inner Ear ◽  
Hair Cells ◽  
Planar Polarity ◽  
Ear Development ◽  
Vestibular Hair Cells ◽  
Inner Ear Development

scholarly journals Cross-regulation of Ngn1 and Math1 coordinates the production of neurons and sensory hair cells during inner ear development

Development ◽  
2007 ◽  
Vol 134 (24) ◽  
pp. 4405-4415 ◽  
Author(s):  
S. Raft ◽  
E. J. Koundakjian ◽  
H. Quinones ◽  
C. S. Jayasena ◽  
L. V. Goodrich ◽  
...  
Keyword(s):  
Inner Ear ◽  
Hair Cells ◽  
Sensory Hair ◽  
Ear Development ◽  
Inner Ear Development ◽  
Sensory Hair Cells

scholarly journals Fgf8 genetic labeling reveals the early specification of vestibular hair cell type in mouse utricle

Development ◽  
2020 ◽  
Vol 147 (22) ◽  
pp. dev192849
Author(s):  
Evan M. Ratzan ◽  
Anne M. Moon ◽  
Michael R. Deans
Keyword(s):  
Hair Cell ◽  
Inner Ear ◽  
Hair Cells ◽  
Conditional Knockout ◽  
Developmental Model ◽  
Type I ◽  
Ear Development ◽  
Inner Ear Development ◽  
Morphological Criteria ◽  
Genetic Labeling

ABSTRACTFGF8 signaling plays diverse roles in inner ear development, acting at multiple stages from otic placode induction to cellular differentiation in the organ of Corti. As a secreted morphogen with diverse functions, Fgf8 expression is likely to be spatially restricted and temporally dynamic throughout inner ear development. We evaluated these characteristics using genetic labeling mediated by Fgf8mcm gene-targeted mice and determined that Fgf8 expression is a specific and early marker of Type-I vestibular hair cell identity. Fgf8mcm expression initiates at E11.5 in the future striolar region of the utricle, labeling hair cells following EdU birthdating, and demonstrates that sub-type identity is determined shortly after terminal mitosis. This early fate specification is not apparent using markers or morphological criteria that are not present before birth in the mouse. Although analyses of Fgf8 conditional knockout mice did not reveal developmental phenotypes, the restricted pattern of Fgf8 expression suggests that functionally redundant FGF ligands may contribute to vestibular hair cell differentiation and supports a developmental model in which Type-I and Type-II hair cells develop in parallel rather than from an intermediate precursor.

scholarly journals Conserved and Divergent Principles of Planar Polarity Revealed by Hair Cell Development and Function

2021 ◽  
Vol 15 ◽  
Author(s):  
Michael R. Deans
Keyword(s):  
Hair Cell ◽  
Inner Ear ◽  
Hair Cells ◽  
Model Organism ◽  
Vestibular Function ◽  
Sensory Receptor ◽  
Apical Surface ◽  
Planar Polarity ◽  
Vestibular Hair Cells ◽  
Sensory Epithelia

Planar polarity describes the organization and orientation of polarized cells or cellular structures within the plane of an epithelium. The sensory receptor hair cells of the vertebrate inner ear have been recognized as a preeminent vertebrate model system for studying planar polarity and its development. This is principally because planar polarity in the inner ear is structurally and molecularly apparent and therefore easy to visualize. Inner ear planar polarity is also functionally significant because hair cells are mechanosensors stimulated by sound or motion and planar polarity underlies the mechanosensory mechanism, thereby facilitating the auditory and vestibular functions of the ear. Structurally, hair cell planar polarity is evident in the organization of a polarized bundle of actin-based protrusions from the apical surface called stereocilia that is necessary for mechanosensation and when stereociliary bundle is disrupted auditory and vestibular behavioral deficits emerge. Hair cells are distributed between six sensory epithelia within the inner ear that have evolved unique patterns of planar polarity that facilitate auditory or vestibular function. Thus, specialized adaptations of planar polarity have occurred that distinguish auditory and vestibular hair cells and will be described throughout this review. There are also three levels of planar polarity organization that can be visualized within the vertebrate inner ear. These are the intrinsic polarity of individual hair cells, the planar cell polarity or coordinated orientation of cells within the epithelia, and planar bipolarity; an organization unique to a subset of vestibular hair cells in which the stereociliary bundles are oriented in opposite directions but remain aligned along a common polarity axis. The inner ear with its complement of auditory and vestibular sensory epithelia allows these levels, and the inter-relationships between them, to be studied using a single model organism. The purpose of this review is to introduce the functional significance of planar polarity in the auditory and vestibular systems and our contemporary understanding of the developmental mechanisms associated with organizing planar polarity at these three cellular levels.

The Effect of Tgfß-2 on Inner Ear Development: Light and Electron Microscopy Observations

1997 ◽  
Vol 3 (S2) ◽  
pp. 173-174
Author(s):  
R. Friedman ◽  
N. Paradies ◽  
S. Wert ◽  
T. Doetschman ◽  
E.L. Cardell
Keyword(s):  
Inner Ear ◽  
Hair Cells ◽  
Transforming Growth Factor Beta ◽  
Transforming Growth Factor ◽  
Light And Electron Microscopy ◽  
Membranous Labyrinth ◽  
Ear Development ◽  
Inner Ear Development

Transforming growth factor beta (TGFß) genes are linked to a variety of developmental processes and are the subject of in vivo and in vitro transgene research studies. We are evaluating TGFß-2 effects on mouse inner ear development, with emphasis on the cochlear duct (CD), by comparing plastic sections of intact inner ears from developmental day (D) 16.5,18.5 and 19.5 littermates with wildtype (+/+), heterozygous (+/−) and mutant (−/−) TGFß-2 genotypes as determined by polymerase chain reaction analysis of tail digests. Auditory and vestibular organs of all D16.5 mice appear similar: membranous labyrinth epithelium varies from simple cuboidal/low columnar to pseudostratified/stratified columnar. Surrounding mesenchyme varies in cell density regionally, the most cellular mesenchyme underlies areas of sensory epithelium. Sparse mesenchymal cell distribution in the vestibule and basal CD indicates sites of perilymph channel formation. The spiral and vestibular ganglia and their unmyelinated fibers are prominent. Otoconia and hair cells are present in the utricle (U) and saccule (S) maculae; hair cells are less easily identifiable in the CD.

scholarly journals Dlx3b/4b is required for early-born but not later-forming sensory hair cells during zebrafish inner ear development

Biology Open ◽  
2017 ◽  
Vol 6 (9) ◽  
pp. 1270-1278 ◽  
Author(s):  
Simone Schwarzer ◽  
Sandra Spieß ◽  
Michael Brand ◽  
Stefan Hans
Keyword(s):  
Inner Ear ◽  
Hair Cells ◽  
Sensory Hair ◽  
Ear Development ◽  
Inner Ear Development ◽  
Sensory Hair Cells

Comparative analysis of conservation and regulatory network on core transcription factors in mouse inner ear development

2013 ◽  
Vol 35 (10) ◽  
pp. 1198-1208
Author(s):  
Zhi-Qiang CHEN ◽  
Xin-Huan HAN ◽  
Qin-Jun WEI ◽  
Guang-Qian XING ◽  
Xin CAO
Keyword(s):  
Transcription Factors ◽  
Comparative Analysis ◽  
Inner Ear ◽  
Regulatory Network ◽  
Ear Development ◽  
Inner Ear Development

scholarly journals Inner Ear and Muscle Developmental Defects in Smpx-Deficient Zebrafish Embryos

2021 ◽  
Vol 22 (12) ◽  
pp. 6497
Author(s):  
Anna Ghilardi ◽  
Alberto Diana ◽  
Renato Bacchetta ◽  
Nadia Santo ◽  
Miriam Ascagni ◽  
...  
Keyword(s):  
Hearing Loss ◽  
Inner Ear ◽  
Hair Cells ◽  
Muscle Fibers ◽  
Underlying Mechanism ◽  
Loss Of Function ◽  
Zebrafish Model ◽  
Developmental Defects ◽  
Inner Ear Development ◽  
Syndromic Hearing Loss

The last decade has witnessed the identification of several families affected by hereditary non-syndromic hearing loss (NSHL) caused by mutations in the SMPX gene and the loss of function has been suggested as the underlying mechanism. In the attempt to confirm this hypothesis we generated an Smpx-deficient zebrafish model, pointing out its crucial role in proper inner ear development. Indeed, a marked decrease in the number of kinocilia together with structural alterations of the stereocilia and the kinocilium itself in the hair cells of the inner ear were observed. We also report the impairment of the mechanotransduction by the hair cells, making SMPX a potential key player in the construction of the machinery necessary for sound detection. This wealth of evidence provides the first possible explanation for hearing loss in SMPX-mutated patients. Additionally, we observed a clear muscular phenotype consisting of the defective organization and functioning of muscle fibers, strongly suggesting a potential role for the protein in the development of muscle fibers. This piece of evidence highlights the need for more in-depth analyses in search for possible correlations between SMPX mutations and muscular disorders in humans, thus potentially turning this non-syndromic hearing loss-associated gene into the genetic cause of dysfunctions characterized by more than one symptom, making SMPX a novel syndromic gene.

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