scholarly journals Loss of Fgfr3 leads to excess hair cell development in the mouse organ of Corti

2007 ◽  
Vol 236 (2) ◽  
pp. 525-533 ◽  
Author(s):  
Toshinori Hayashi ◽  
Dale Cunningham ◽  
Olivia Bermingham-McDonogh
Keyword(s):  
Hair Cell ◽  
Organ Of Corti ◽  
Cell Development ◽  
Mouse Organ

scholarly journals Sox2 and FGF20 interact to regulate organ of Corti hair cell and supporting cell development in a spatially-graded manner

2018 ◽  
Author(s):  
Lu M. Yang ◽  
Kathryn S.E. Cheah ◽  
Sung-Ho Huh ◽  
David M. Ornitz
Keyword(s):  
Hearing Loss ◽  
Hair Cell ◽  
Hair Cells ◽  
Genetic Interaction ◽  
Organ Of Corti ◽  
Supporting Cell ◽  
Cell Development ◽  
Supporting Cells ◽  
Outer Compartment ◽  
Mouse Organ

AbstractThe mouse organ of Corti develops in two steps: progenitor specification and differentiation. Fibroblast Growth Factor (FGF) signaling is important in this developmental pathway, as deletion of FGF receptor 1 (Fgfr1) or its ligand, Fgf20, leads to the loss of hair cells and supporting cells from the organ of Corti. However, whether FGF20-FGFR1 signaling is required during specification or differentiation, and how it interacts with the transcription factor Sox2, also important for hair cell and supporting cell development, has been a topic of debate. Here, we show that while FGF20-FGFR1 signaling functions during progenitor differentiation, FGFR1 has an FGF20-independent, Sox2-dependent role in specification. We also show that a combination of reduction in Sox2 expression and Fgf20 deletion recapitulates the Fgfr1-deletion phenotype. Furthermore, we uncovered a strong genetic interaction between Sox2 and Fgf20, especially in regulating the development of hair cells and supporting cells towards the basal end and the outer compartment of the organ of Corti. To explain this genetic interaction and its effects on the basal end of the organ of Corti, we provide evidence that decreased Sox2 expression delays specification, which begins at the organ of Corti apex, while Fgf20-deletion results in premature onset of differentiation, which begins near the organ of Corti base. Thereby, Sox2 and Fgf20 interact to ensure that specification occurs before differentiation towards the cochlear base. These findings reveal an intricate developmental program regulating organ of Corti development along the basal-apical axis of the cochlea.Author summaryThe mammalian cochlea contains the organ of Corti, a specialized sensory epithelium populated by hair cells and supporting cells that detect sound. Hair cells are susceptible to injury by noise, toxins, and other insults. In mammals, hair cells cannot be regenerated after injury, resulting in permanent hearing loss. Understanding genetic pathways that regulate hair cell development in the mammalian organ of Corti will help in developing methods to regenerate hair cells to treat hearing loss. Many genes are essential for hair cell and supporting cell development in the mouse organ of Corti. Among these are Sox2, Fgfr1, and Fgf20. Here, we investigate the relationship between these three genes to further define their roles in development.Interestingly, we found that Sox2 and Fgf20 interact to affect hair cell and supporting cell development in a spatially-graded manner. We found that cells toward the outer compartment and the base of the organ of Corti are more strongly affected by the loss of Sox2 and Fgf20. We provide evidence that this spatially-graded effect can be partially explained by the roles of the two genes in the precise timing of two sequential stages of organ of Corti development, specification and differentation.

scholarly journals Sox2 and FGF20 interact to regulate organ of Corti hair cell and supporting cell development in a spatially-graded manner

PLoS Genetics ◽  
2019 ◽  
Vol 15 (7) ◽  
pp. e1008254 ◽  
Author(s):  
Lu M. Yang ◽  
Kathryn S. E. Cheah ◽  
Sung-Ho Huh ◽  
David M. Ornitz
Keyword(s):  
Hair Cell ◽  
Organ Of Corti ◽  
Supporting Cell ◽  
Cell Development

scholarly journals THE ULTRASTRUCTURE OF THE SENSORY HAIRS AND ASSOCIATED ORGANELLES OF THE COCHLEAR INNER HAIR CELL, WITH REFERENCE TO DIRECTIONAL SENSITIVITY

1966 ◽  
Vol 29 (3) ◽  
pp. 497-505 ◽  
Author(s):  
Arndt J. Duvall ◽  
Åke Flock ◽  
Jan Wersäll
Keyword(s):  
Guinea Pig ◽  
Hair Cell ◽  
Basal Body ◽  
Organ Of Corti ◽  
Sensory Cells ◽  
Directional Sensitivity ◽  
Inner Hair Cell ◽  
Lateral Line Organ ◽  
Sensory Hairs ◽  
Line Organ

From the apical end of the inner hair cell of the organ of Corti in the guinea pig cochlea protrude four to five rows of stereocilia shaped in a pattern not unlike the wings of a bird. In the area devoid of cuticular substance facing toward the tunnel of Corti lies a consistently present centriole. The ultrastructure of this centriole is similar to that of the basal body of the kinocilium located in the periphery of the sensory hair bundles in the vestibular and lateral line organ sensory cells and to that of the centrioles of other cells. The physiological implications of the anatomical orientation of this centriole are discussed in terms of directional sensitivity.

Gene Expression Profile of the Mouse Organ of Corti at the Onset of Hearing

Genomics ◽  
2004 ◽  
Vol 83 (6) ◽  
pp. 1000-1011 ◽  
Author(s):  
Celine Pompeia ◽  
Belen Hurle ◽  
Inna A. Belyantseva ◽  
Konrad Noben-Trauth ◽  
Kirk Beisel ◽  
...  
Keyword(s):  
Gene Expression ◽  
Gene Expression Profile ◽  
Expression Profile ◽  
Organ Of Corti ◽  
Mouse Organ

Shaker-1 mutations reveal roles for myosin VIIA in both development and function of cochlear hair cells

Development ◽  
1998 ◽  
Vol 125 (4) ◽  
pp. 557-566 ◽  
Author(s):  
T. Self ◽  
M. Mahony ◽  
J. Fleming ◽  
J. Walsh ◽  
S.D. Brown ◽  
...  
Keyword(s):  
Hair Cell ◽  
Hair Cells ◽  
Usher Syndrome ◽  
Orthologous Gene ◽  
Cell Development ◽  
Cochlear Hair Cells ◽  
Cochlear Hair Cell ◽  
Show Evidence ◽  
Myosin Viia ◽  
And Function

The mouse shaker-1 locus, Myo7a, encodes myosin VIIA and mutations in the orthologous gene in humans cause Usher syndrome type 1B or non-syndromic deafness. Myo7a is expressed very early in sensory hair cell development in the inner ear. We describe the effects of three mutations on cochlear hair cell development and function. In the Myo7a816SB and Myo7a6J mutants, stereocilia grow and form rows of graded heights as normal, but the bundles become progressively more disorganised. Most of these mutants show no gross electrophysiological responses, but some did show evidence of hair cell depolarisation despite the disorganisation of their bundles. In contrast, the original shaker-1 mutants, Myo7ash1, had normal early development of stereocilia bundles, but still showed abnormal cochlear responses. These findings suggest that myosin VIIA is required for normal stereocilia bundle organisation and has a role in the function of cochlear hair cells.

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