Abstract
The inner ear develops from a simple epithelium (otic placode) into the complex structures specialized for balance (vestibule) and sound (cochlea) detection. Abnormal vestibular and cochlear development is associated with many birth defects. During recent years, considerable progress has been made in understanding the molecular bases of these conditions. To determine the biological function of two closely related GTPase activating proteins for the Cdc42/Rac GTPases, Abr and Bcr, we generated a mouse strain deficient in both of these proteins. Double null mutant mice exhibit hyperactivity, persistent circling, and are unable to swim. These phenotypes are typically found in mice with vestibular defects. Indeed, adult double null mutants display abnormal dysmorphic structures of both the saccule and utricle. Moreover, a total loss of otoconia can be seen in the utricle, whereas in the saccule, otoconia are either missing or their number is drastically decreased and they are abnormally large. Interestingly, both the cochlea and semicircular canals are normal and the double null mutant mice are not deaf. These data demonstrate that Abr and Bcr play important complementary roles during vestibular morphogenesis and that a function of ...Continue Reading
References
May 22, 1992·Brain Research. Developmental Brain Research·T J MahalikG Clayton
May 1, 1992·Molecular Biology of the Cell·A Hall
Jan 1, 1971·Genetics·L C ErwayL S Hurley
Oct 24, 1995·Proceedings of the National Academy of Sciences of the United States of America·T H ChuangG M Bokoch
Mar 2, 1995·Nature·D WeilM D Weston
Mar 2, 1995·Nature·F GibsonS D Brown
Oct 6, 1997·Genes & Development·L Van Aelst, C D'Souza-Schorey
Nov 21, 1997·Science·E D LynchM C King
Jun 20, 1998·Mechanisms of Development·M Torres, F Giráldez
Oct 2, 1998·Genetics·R A BergstromJ C Schimenti
Jan 20, 1999·Proceedings of the National Academy of Sciences of the United States of America·E VerpyC Petit
Aug 3, 1999·Genetics·K N AlagramamR P Woychik
Aug 17, 1999·Current Opinion in Neurobiology·R Kollmar
Jan 4, 2001·Nature Genetics·K N AlagramamR P Woychik
Citations
Sep 23, 2003·Developmental Biology·Bruce B Riley, Bryan T Phillips
Jun 2, 2011·European Journal of Human Genetics : EJHG·Christine M ArmourGail E Graham
Aug 14, 2013·The Journal of Cell Biology·Adi D DubashKathleen J Green
Aug 26, 2009·Molecular and Cellular Biology·Jess M CunnickNora Heisterkamp
Nov 23, 2006·Molecular and Cellular Biology·Young Jin ChoNora Heisterkamp
Jun 1, 2012·PloS One·Nichol L G MillerDavid D Schlaepfer
Sep 17, 2013·Oncogene·D R CookC J Der
Dec 4, 2012·European Journal of Medical Genetics·Florence PetitMichèle Mathieu-Dramard
May 3, 2011·Progress in Neurobiology·Kimberley F ToliasKyongmi Um
Sep 8, 2010·Journal of Neuroscience Methods·Chris R S KanekoJohn Yarno
Jun 25, 2014·Developmental Cell·Kyongmi UmKimberley F Tolias
Mar 23, 2013·Small GTPases·Roland Csépányi-KömiErzsébet Ligeti
Mar 15, 2014·The Cleft Palate-craniofacial Journal : Official Publication of the American Cleft Palate-Craniofacial Association·Rita M IbitoyeUsha Kini
Jun 28, 2014·Neuroscience and Biobehavioral Reviews·Bianca De FilippisGiovanni Laviola
Aug 9, 2003·American Journal of Human Genetics·Jessica YinglingAnthony Wynshaw-Boris
Feb 12, 2015·PloS One·Pai-Tsang HuangYung-Feng Lin
Nov 16, 2012·PloS One·Min YuNora Heisterkamp
Feb 3, 2005·Nature Reviews. Molecular Cell Biology·Kent L RossmanJohn Sondek
Dec 12, 2007·The Journal of Biological Chemistry·Soo-Mi KweonNora Heisterkamp
Feb 3, 2012·Physiological Reviews·Erzsébet LigetiKlaus Scheffzek
Sep 26, 2002·FEBS Letters·Jeremy PeckPeter D Burbelo
Jun 17, 2006·Cancer Research·Oyenike O OlabisiIan P Whitehead
May 28, 2015·Clinical Cancer Research : an Official Journal of the American Association for Cancer Research·Masaaki KomatsuM Eileen Dolan
Jul 1, 2021·Neurogastroenterology and Motility : the Official Journal of the European Gastrointestinal Motility Society·Yongtao XiaoYing Wang