Cochlear homeostasis: a molecular physiological perspective on maintenance of sound transduction and auditory neurotransmission with noise and ageing

2020 ◽  
Vol 18 ◽  
pp. 106-115
Author(s):  
Gary D Housley ◽  
Georg von Jonquieres ◽  
Jeremy L Pinyon ◽  
Julie-Anne T Matheson ◽  
Lily J Pearson ◽  
...  
Keyword(s):  
2021 ◽  
pp. 2107167
Author(s):  
Kaiwen Hu ◽  
William Cardenas ◽  
Yi‐Chi Huang ◽  
Huijing Wei ◽  
Robert‐Eric Gaskell ◽  
...  

2002 ◽  
Vol 7 (1) ◽  
pp. 55-61 ◽  
Author(s):  
G.D. Housley ◽  
D.J. Jagger ◽  
D. Greenwood ◽  
N.P. Raybould ◽  
S.G. Salih ◽  
...  
Keyword(s):  

2011 ◽  
Author(s):  
Manuela Nowotny ◽  
Arun Palghat Udayashankar ◽  
Melanie Weber ◽  
Jennifer Hummel ◽  
Manfred Kössl ◽  
...  

Development ◽  
2021 ◽  
Author(s):  
Amandine Jarysta ◽  
Basile Tarchini

Sound transduction occurs in the hair bundle, the apical compartment of sensory hair cells in the inner ear. The hair bundle is formed of actin-based stereocilia aligned in rows of graded heights. It was previously shown that the GNAI-GPSM2 complex is part of a developmental blueprint that defines the polarized organization of the apical cytoskeleton in hair cells, including stereocilia distribution and elongation. Here we report a novel and critical role for Multiple PDZ domain (MPDZ) protein during apical hair cell morphogenesis. We show that MPDZ is enriched at the hair cell apical membrane along with MAGUK p55 subfamily member 5 (MPP5/PALS1) and the Crumbs protein CRB3. MPDZ is required there to maintain the proper segregation of apical blueprints proteins, including GNAI-GPSM2. Loss of the blueprint coincides with misaligned stereocilia placement in Mpdz mutant hair cells, and results in permanently misshapen hair bundles. Graded molecular and structural defects along the cochlea can explain the profile of hearing loss in Mpdz mutants, where deficits are most severe at high frequencies.


1985 ◽  
Vol 78 (5) ◽  
pp. 1508-1518 ◽  
Author(s):  
J. L. Flanagan ◽  
J. D. Johnston ◽  
R. Zahn ◽  
G. W. Elko

2010 ◽  
Vol 263 (1-2) ◽  
pp. 104-113 ◽  
Author(s):  
Rodney Perkins ◽  
Jonathan P. Fay ◽  
Paul Rucker ◽  
Micha Rosen ◽  
Lisa Olson ◽  
...  
Keyword(s):  

1985 ◽  
Vol 78 (S1) ◽  
pp. S52-S52 ◽  
Author(s):  
J. L. Flanagan ◽  
J. D. Johnston ◽  
R. Zahn ◽  
G. W. Elko

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