Auditory function in the Tc1 mouse model of down syndrome suggests a limited region of human chromosome 21 involved in otitis media.

PloS One
Stephanie KuhnWalter Marcotti

Abstract

Down syndrome is one of the most common congenital disorders leading to a wide range of health problems in humans, including frequent otitis media. The Tc1 mouse carries a significant part of human chromosome 21 (Hsa21) in addition to the full set of mouse chromosomes and shares many phenotypes observed in humans affected by Down syndrome with trisomy of chromosome 21. However, it is unknown whether Tc1 mice exhibit a hearing phenotype and might thus represent a good model for understanding the hearing loss that is common in Down syndrome. In this study we carried out a structural and functional assessment of hearing in Tc1 mice. Auditory brainstem response (ABR) measurements in Tc1 mice showed normal thresholds compared to littermate controls and ABR waveform latencies and amplitudes were equivalent to controls. The gross anatomy of the middle and inner ears was also similar between Tc1 and control mice. The physiological properties of cochlear sensory receptors (inner and outer hair cells: IHCs and OHCs) were investigated using single-cell patch clamp recordings from the acutely dissected cochleae. Adult Tc1 IHCs exhibited normal resting membrane potentials and expressed all K(+) currents characteristic of control hair cells....Continue Reading

References

Nov 5, 1982·Science·P DallosA Flock
Oct 1, 1995·Nature Genetics·R H ReevesM T Davisson
Mar 1, 1996·Archives of Otolaryngology--head & Neck Surgery·H BilginC T Le
Nov 9, 2001·International Journal of Pediatric Otorhinolaryngology·S R ShottD Heithaus
Aug 26, 2003·The Journal of Physiology·Walter MarcottiCorne J Kros
Jun 24, 2004·Nature Reviews. Genetics·Lin He, Gregory J Hannon
Sep 24, 2005·Science·Aideen O'DohertyElizabeth M C Fisher
Dec 24, 2005·Nature Reviews. Neuroscience·Robert Fettiplace, Carole M Hackney
Aug 15, 2006·Brain Research·Michael D WestonGarrett A Soukup
Feb 14, 2008·Nature Reviews. Molecular Cell Biology·Giovanni Stefani, Frank J Slack
Apr 5, 2008·Biochemical and Biophysical Research Communications·Donald E KuhnTerry S Elton
Jan 7, 2009·The Journal of Physiology·Michelle E OlahJohn F MacDonald
Feb 25, 2009·The Journal of Physiology·Stuart L JohnsonWalter Marcotti
Mar 20, 2009·Human Molecular Genetics·Frances K WisemanElizabeth M C Fisher
Apr 14, 2009·Nature Genetics·Morag A LewisKaren P Steel
Apr 14, 2009·Nature Genetics·Angeles MencíaMiguel Angel Moreno-Pelayo
Jul 29, 2009·Genetics in Medicine : Official Journal of the American College of Medical Genetics·André MégarbanéWilliam C Mobley
Sep 22, 2009·International Journal of Experimental Pathology·Fengchan HanQing Y Zheng
Nov 13, 2009·The Journal of Neuroscience : the Official Journal of the Society for Neuroscience·Lely A QuinaEric E Turner
Feb 16, 2010·Blood·Kate A AlfordVictor L J Tybulewicz
Jun 19, 2010·Cardiovascular Research·Louisa DunlevyTimothy Mohun
Jan 20, 2011·Proceedings of the National Academy of Sciences of the United States of America·Stephanie KuhnWalter Marcotti
Mar 15, 2011·Mammalian Genome : Official Journal of the International Mammalian Genome Society·Xiaolu Sturgeon, Katheleen J Gardiner

❮ Previous
Next ❯

Citations

Jan 26, 2013·Human Molecular Genetics·Md Mahiuddin AhmedKatheleen J Gardiner
Sep 27, 2013·Mammalian Genome : Official Journal of the International Mammalian Genome Society·Mahmood F BhuttaSteve D M Brown
Oct 30, 2012·Gene·Claire SpellmanKatheleen J Gardiner
Apr 5, 2017·Otolaryngology--head and Neck Surgery : Official Journal of American Academy of Otolaryngology-Head and Neck Surgery·Jizhen LinYan Zhang

❮ Previous
Next ❯

Methods Mentioned

BETA
dissecting
genotyping

Software Mentioned

custom
pClamp
Origin

Related Concepts

Related Feeds

Cajal Bodies & Gems

Cajal bodies or coiled bodies are dense foci of coilin protein. Gemini of Cajal bodies, or gems, are microscopically similar to Cajal bodies. It is believed that Cajal bodies play important roles in RNA processing while gems assist the Cajal bodies. Find the latest research on Cajal bodies and gems here.

Birth Defects

Birth defects encompass structural and functional alterations that occur during embryonic or fetal development and are present since birth. The cause may be genetic, environmental or unknown and can result in physical and/or mental impairment. Here is the latest research on birth defects.

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.

Related Papers

Proceedings of the National Academy of Sciences of the United States of America
Stephanie KuhnWalter Marcotti
Mammalian Genome : Official Journal of the International Mammalian Genome Society
Mahmood F BhuttaSteve D M Brown
Proceedings of the National Academy of Sciences of the United States of America
Stuart L JohnsonWalter Marcotti
© 2022 Meta ULC. All rights reserved