Abstract
Loss-of function mutations in transmembrane inner ear expressed (Tmie/TMIE) gene have been shown to cause deafness in mice and humans (DFNB6). However, the functional roles of TMIE in the cochlea remain unclear. A primary step toward the understanding of the role of TMIE in hearing and its dysfunction is the documentation of its cellular and sub-cellular location within the cochlea, the auditory organ. In this study, we located and determined the cellular expression of Tmie within the rat cochlea using a polyclonal anti-Tmie antibody. The anti-Tmie antibody identified a specific band of 17 kDa in a variety of rat tissues by using Western blot analyses. The expression products of Tmie were also detected in the spiral limbus, spiral ligament, organ of Corti, and stria vascularis by immunohistochemistry analysis and RT-PCR. Our results point out the presence and localization of Tmie products in the cochlea of rat. Knowledge of spatial distribution of Tmie will provide important insight into the mechanisms that lead to deafness due to mutations in the TMIE gene.
Similar content being viewed by others
Abbreviations
- Tmie/TMIE :
-
Transmembrane inner ear expressed
- RT-PCR:
-
Reverse transcription and polymerase chain reaction
- DFNA:
-
Deafness, autosomal dominant nonsyndromic
- DFNB:
-
Deafness, autosomal recessive nonsyndromic
- PCR:
-
Polymerase chain reaction
- NSHI:
-
Nonsyndromic hearing impaired
- LCM:
-
Laser capture microdissection
- OC:
-
Organ of Corti.
- IHC:
-
Inner hair cell
- OHC:
-
Outer hair cell
- SLim:
-
Spiral limbus
- SLig:
-
Spiral ligament
- BM:
-
Basilar membrane
- SV:
-
Stria vascularis
- TM:
-
Tectorial membrane
References
Bakker C, Verheij C, Willemsen R, Van Der Helm R (1994) Fmr1 knockout mice: a model to study fragile X mental retardation. The Dutch-Belgian fragile X consortium. Cell 78:23–33
Henson MM, Burridge K, Fitzpatrick D, Jenkins DB, Pillsbury HC, Henson OW Jr (1985) Immunocytochemical localization of contractile and contraction associated proteins in the spiral ligament of the cochlea. Hear Res 20:207–214
Kachar B, Battaglia A, Fex J (1997) Compartmentalized vesicular traffic around the hair cell cuticular plate. Hear Res 107:102–112
Konishi T, Harick DE, Walsh PJ (1978) Ion transport in guinea pig cochlea. I. Potassium and sodium transport. Acta Otolaryngol 86:22–34
Kooy RF, Willemsen R, Oostra BA (2000) Fragile X syndrome at the turn of the century. Mol Med Today 6:193–198
Krogh A, Larsson B, von Heijne G, Sonnhammer EL (2001) Predicting transmembrane protein topology with a hidden Markov model: application to complete genomes. J Mol Biol 305:567–580
Lalwani AK, Castelein CM, Cracking the Auditory Genetic Code (1999) Nonsyndromic hereditary hearing impairment. Am J Otol 20:115–132
Mhatre AN, Li Y, Atkin G, Maghnouj A, Lalwani AK (2006) Expression of Myh9 in the mammalian cochlea: localization within the stereocilia. J Neuro Res 84:809–818
Minowa O, Ikeda K, Sugitani Y, Oshima T, Nakai S, Katori Y, Suzuki M, Furukawa M, Kawase T, Zheng Y, Ogura M, Asada Y, Watanabe K, Yamanaka H, Gotoh S, Nishi-Takeshima M, Sugimoto T, Kikuchi T, Takasaka T, Noda T (1999) Altered cochlear fibrocytes in a mouse model of DFN3 nonsyndromic deafness. Science 5432:1408–1411
Mitchem KL, Hibbard E, Beyer LA, Bosom K, Dootz GA, Dolan DF, Johnson KR, Raphael Y, Kohrman DC (2002) Mutation of the novel gene Tmie results in sensory cell defects in the inner ear of spinner, a mouse model of human hearing loss DFNB6. Hum Mol Genet 16:1887–1898
Morton CC (2002) Genetics, genomics and gene discovery in the auditory system. Hum Mol Genet 11:1229–1240
Morton NE (1991) Genetic epidemiogy of hearing impairment. Ann NY Acad Sci 630:16–31
Naz S, Giguere CM, Kohrman DC, Mitchem KL, Riazuddin S, Morell RJ, Ramesh A, Srisailpathy S, Deshmukh D, Riazuddin S, Griffith AJ, Friedman TB, Smith RJH, Wilcox ER (2002) Mutations in a novel gene, TMIE, are associated with hearing loss linked to the DFNB6 locus. Am J Hum Genet 71:632–636
Pickles JO, Comis SD, Osborne MP (1984) Cross-links between stereocilia in the guinea pig organ of Corti, and their possible relation to sensory transduction. Hear Res 15:103–112
Raphael Y, Athey BD, Wang Y, Lee MK, Altschuler RA (1994) F-actin, tubulin and spectrin in the organ of Corti: comparative distribution in different cell types and mammalian species. Hear Res 76:173–187
Richardson GP, Russell IJ, Duance VC, Bailey AJ (1987) Polypeptide composition of the mammalian tectorial membrane. Hear Res 25:45–60
Spicer SS, Schulte BA (1998) Evidence for a medial K+ recycling pathway from inner hair cell. Hear Res 118:1–12
Thorne PR, Carlisle L, Zajic G, Schacht J, Altschuler RA (1987) Differences in the distribution of F-actin in outer hair cells along the organ of Corti. Hear Res 30:253–265
Ulfendahl M, Flock A, Scarfone E (2001) Structural relationships of the unfixed tectorial membrane. Hear Res 151(1–2):41–47
van Camp G, Smith RJH (2006) Hereditary hearing loss homepage. http://webh01.ua.ac.be/hhh/
Acknowledgments
We thank Dr. Seng-Sheen Fan (Department of Life science, Tunghai University) for providing the Zeiss LSM510 inverted confocal microscope and for technical assistance in this study. This work was supported by the National Science Council, Republic of China (NSC93-2320-B-040-055 and NSC94-2320-B-040-043)and Chung-Shan Medical University Hospital (CSH-97-B-08).
Author information
Authors and Affiliations
Corresponding author
Rights and permissions
About this article
Cite this article
Su, MC., Yang, JJ., Chou, MY. et al. Expression and localization of Tmie in adult rat cochlea. Histochem Cell Biol 130, 119–126 (2008). https://doi.org/10.1007/s00418-008-0385-z
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s00418-008-0385-z