Tprn is essential for the integrity of stereociliary rootlet in cochlear hair cells in mice

Frontiers of Medicine
Yuqin MenJiangang Gao

Abstract

Tprn encodes the taperin protein, which is concentrated in the tapered region of hair cell stereocilia in the inner ear. In humans, TPRN mutations cause autosomal recessive nonsyndromic deafness (DFNB79) by an unknown mechanism. To determine the role of Tprn in hearing, we generated Tprn-null mice by clustered regularly interspaced short palindromic repeat/Cas9 genome-editing technology from a CBA/CaJ background. We observed significant hearing loss and progressive degeneration of stereocilia in the outer hair cells of Tprn-null mice starting from postnatal day 30. Transmission electron microscopy images of stereociliary bundles in the mutant mice showed some stereociliary rootlets with curved shafts. The central cores of the stereociliary rootlets possessed hollow structures with surrounding loose peripheral dense rings. Radixin, a protein expressed at stereocilia tapering, was abnormally dispersed along the stereocilia shafts in Tprn-null mice. The expression levels of radixin and β-actin significantly decreased.We propose that Tprn is critical to the retention of the integrity of the stereociliary rootlet. Loss of Tprn in Tprn-null mice caused the disruption of the stereociliary rootlet, which resulted in damage to stereocil...Continue Reading

References

Jan 1, 1991·Annual Review of Neuroscience·J T Corwin, M E Warchol
Dec 1, 1980·The Journal of Cell Biology·L G Tilney, L A Jaffe
Jun 16, 1997·The Journal of Cell Biology·T HassonD P Corey
Feb 26, 2003·Cell·Thomas D Pollard, Gary G Borisy
Feb 26, 2004·Proceedings of the National Academy of Sciences of the United States of America·F PatakyA J Hudspeth
Jun 24, 2004·Nature Reviews. Genetics·Gregory I FrolenkovAndrew J Griffith
Aug 18, 2004·The Journal of Cell Biology·Shin-ichiro KitajiriSachiko Tsukita
Mar 19, 2005·The Journal of Comparative Neurology·Richard J GoodyearGuy P Richardson
Oct 6, 2006·The Journal of Neuroscience : the Official Journal of the Society for Neuroscience·Leona H GagnonKenneth R Johnson
Jul 6, 2007·Hearing Research·Qing Yin ZhengHeping Yu
May 28, 2009·Nature Neuroscience·Christine Petit, Guy P Richardson
Jul 16, 2009·European Journal of Human Genetics : EJHG·Shahid Yar KhanThomas B Friedman
Dec 4, 2009·The Journal of Neuroscience : the Official Journal of the Society for Neuroscience·Anthony W PengStefan Heller
Feb 23, 2010·American Journal of Human Genetics·Yun LiBernd Wollnik
Jun 1, 2010·Cell·Jacques Boutet de Monvel, Christine Petit
Aug 26, 2011·The Journal of Neuroscience : the Official Journal of the Society for Neuroscience·Katherine A SteigelmanJian Zuo
Dec 28, 2011·Hearing Research·Meghan C DrummondThomas B Friedman
May 10, 2013·The Journal of Neuroscience : the Official Journal of the Society for Neuroscience·Benjamin J PerrinJames M Ervasti
Aug 7, 2013·Proceedings of the National Academy of Sciences of the United States of America·David N FurnessWalter Marcotti
Jul 6, 2014·Physiological Reviews·Robert Fettiplace, Kyunghee X Kim
Nov 16, 2014·Methods in Enzymology·Yoshitaka Fujihara, Masahito Ikawa
Feb 6, 2015·The Journal of Neuroscience : the Official Journal of the Society for Neuroscience·Shimon P FrancisJung-Bum Shin
Mar 21, 2015·Nature Reviews. Drug Discovery·Ulrich Müller, Peter G Barr-Gillespie
Oct 4, 2016·Biochemical and Biophysical Research Communications·Mo ChenDing-Hua Xie

❮ Previous
Next ❯

Citations

Jul 8, 2020·Journal of Medical Genetics·Suzanne E de BruijnUNKNOWN DOOFNL Consortium
Jan 18, 2020·International Journal of Molecular Sciences·Itallia PacentinePeter G Barr-Gillespie

❮ Previous
Next ❯

Related Concepts

Related Feeds

CRISPR Ribonucleases Deactivation

CRISPR-Cas system enables the editing of genes to create or correct mutations. This feed focuses on mechanisms that underlie deactivation of CRISPR ribonucleases. Here is the latest research.

Auditory Perception

Auditory perception is the ability to receive and interpret information attained by the ears. Here is the latest research on factors and underlying mechanisms that influence auditory perception.

CRISPR (general)

Clustered regularly interspaced short palindromic repeats (CRISPR) are DNA sequences in the genome that are recognized and cleaved by CRISPR-associated proteins (Cas). CRISPR-Cas system enables the editing of genes to create or correct mutations. Discover the latest research on CRISPR here.