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Hoffmann, R. A wiki for the life sciences where authorship matters. Nature Genetics (2008)
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Disease relevance of Cochlea


Psychiatry related information on Cochlea

  • An extended region of the greater mustached bat's cochlea, the sparsely innervated (SI) zone, is located just basally to the frequency place of the dominant 61-kHz component of the echolocation signal (CF2) [6].
  • Therefore, 5-HT fibers projecting to the cochlea might be involved, as in other parts of the auditory pathway, in alertness, attention, control of sleep or wakefulness cycles, and state of urgency prior to the transduction processing at the auditory receptor [7].
  • We compared eNOS expression in gentamicin-treated and non-treated guinea pigs in the second turn of the cochlea, an area corresponding to speech perception in humans [8].

High impact information on Cochlea

  • In the mouse cochlea, whirlin is expressed in the sensory IHC and OHC stereocilia [9].
  • In the cochlea, ultrastructural analysis of the TM indicated that otogelin is involved in the organization of its fibrillar network [10].
  • These findings implicate Fkh10 as an early regulator necessary for development of both cochlea and vestibulum and identify its human homologue FKHL10 as a previously unknown candidate deafness gene at 5q34 [11].
  • The auditory-evoked brainstem response (ABR) in Thrb-/- mice, although greatly diminished, displayed normal waveforms, which suggested that the primary defect resides in the cochlea [12].
  • Fibroblast growth factor receptor 3 (Fgfr3) is a tyrosine kinase receptor expressed in developing bone, cochlea, brain and spinal cord [13].

Chemical compound and disease context of Cochlea


Biological context of Cochlea


Anatomical context of Cochlea


Associations of Cochlea with chemical compounds

  • The mouse cochlea expresses type 2 deiodinase (D2), an enzyme that converts thyroxine, the main form of thyroid hormone in the circulation, into 3,5,3'-triiodothyronine (T3) the major ligand for TRs [28].
  • The activity decreased to less than 20% of its initial level after incubation with excess amounts of a polyclonal or a monoclonal antibody against the brain enzyme, but the activity remained unchanged with a polyclonal antibody against the spleen enzyme, indicating that the brain-type enzyme synthesizes prostaglandin D2 in the cochlea [29].
  • Brain-type prostaglandin D synthetase occurs in the rat cochlea [29].
  • The mechanics of the cochlea are vulnerable, and dramatic changes are seen especially when the sensory hair cells are affected, for example, following acoustic overstimulation or exposure to ototoxic compounds such as furosemide [30].
  • In this study cell cycle-associated events in the avian cochlea were analyzed at early and late time intervals following a single high dose of gentamicin [31].

Gene context of Cochlea


Analytical, diagnostic and therapeutic context of Cochlea


  1. Complementary roles of neurotrophin 3 and a N-methyl-D-aspartate antagonist in the protection of noise and aminoglycoside-induced ototoxicity. Duan, M., Agerman, K., Ernfors, P., Canlon, B. Proc. Natl. Acad. Sci. U.S.A. (2000) [Pubmed]
  2. Connexin 31 (GJB3) is expressed in the peripheral and auditory nerves and causes neuropathy and hearing impairment. López-Bigas, N., Olivé, M., Rabionet, R., Ben-David, O., Martínez-Matos, J.A., Bravo, O., Banchs, I., Volpini, V., Gasparini, P., Avraham, K.B., Ferrer, I., Arbonés, M.L., Estivill, X. Hum. Mol. Genet. (2001) [Pubmed]
  3. Expression of cell adhesion molecules during embryonic induction. III. Development of the otic placode. Richardson, G.P., Crossin, K.L., Chuong, C.M., Edelman, G.M. Dev. Biol. (1987) [Pubmed]
  4. Adenovirus-mediated overexpression of a gene prevents hearing loss and progressive inner hair cell loss after transient cochlear ischemia in gerbils. Hakuba, N., Watabe, K., Hyodo, J., Ohashi, T., Eto, Y., Taniguchi, M., Yang, L., Tanaka, J., Hata, R., Gyo, K. Gene Ther. (2003) [Pubmed]
  5. Riluzole rescues cochlear sensory cells from acoustic trauma in the guinea-pig. Wang, J., Dib, M., Lenoir, M., Vago, P., Eybalin, M., Hameg, A., Pujol, R., Puel, J.L. Neuroscience (2002) [Pubmed]
  6. Micromechanical responses to tones in the auditory fovea of the greater mustached bat's cochlea. Russell, I.J., Kössl, M. J. Neurophysiol. (1999) [Pubmed]
  7. Serotonergic innervation of the inner ear: is it involved in the general physiological control of the auditory receptor? Bartolomé, M.V., Gil-Loyzaga, P. The international tinnitus journal. (2005) [Pubmed]
  8. Endothelial nitric oxide synthase upregulation in the cochlea of the guinea pig after intratympanic gentamicin injection. Heinrich, U.R., Selivanova, O., Brieger, J., Mann, W.J. European archives of oto-rhino-laryngology : official journal of the European Federation of Oto-Rhino-Laryngological Societies (EUFOS) : affiliated with the German Society for Oto-Rhino-Laryngology - Head and Neck Surgery. (2006) [Pubmed]
  9. Defects in whirlin, a PDZ domain molecule involved in stereocilia elongation, cause deafness in the whirler mouse and families with DFNB31. Mburu, P., Mustapha, M., Varela, A., Weil, D., El-Amraoui, A., Holme, R.H., Rump, A., Hardisty, R.E., Blanchard, S., Coimbra, R.S., Perfettini, I., Parkinson, N., Mallon, A.M., Glenister, P., Rogers, M.J., Paige, A.J., Moir, L., Clay, J., Rosenthal, A., Liu, X.Z., Blanco, G., Steel, K.P., Petit, C., Brown, S.D. Nat. Genet. (2003) [Pubmed]
  10. Targeted disruption of otog results in deafness and severe imbalance. Simmler, M.C., Cohen-Salmon, M., El-Amraoui, A., Guillaud, L., Benichou, J.C., Petit, C., Panthier, J.J. Nat. Genet. (2000) [Pubmed]
  11. The winged helix transcription factor Fkh10 is required for normal development of the inner ear. Hulander, M., Wurst, W., Carlsson, P., Enerbäck, S. Nat. Genet. (1998) [Pubmed]
  12. Thyroid hormone receptor beta is essential for development of auditory function. Forrest, D., Erway, L.C., Ng, L., Altschuler, R., Curran, T. Nat. Genet. (1996) [Pubmed]
  13. Skeletal overgrowth and deafness in mice lacking fibroblast growth factor receptor 3. Colvin, J.S., Bohne, B.A., Harding, G.W., McEwen, D.G., Ornitz, D.M. Nat. Genet. (1996) [Pubmed]
  14. Ultrastructural changes in the hydropic cochlea of the guinea-pig. Horner, K.C., Guilhaume, A. Eur. J. Neurosci. (1995) [Pubmed]
  15. Effects of nitric oxide on normal and ischemic cochlea of the guinea pig. Ruan, R.S., Leong, S.K., Yeoh, K.H. Exp. Neurol. (2001) [Pubmed]
  16. Noise-induced aspartate and glutamate efflux in the guinea pig cochlea and hearing loss. Jäger, W., Goiny, M., Herrera-Marschitz, M., Brundin, L., Fransson, A., Canlon, B. Experimental brain research. Experimentelle Hirnforschung. Expérimentation cérébrale. (2000) [Pubmed]
  17. Clinical and molecular analysis of three Mexican families with Pendred's syndrome. Gonzalez Trevino, O., Karamanoglu Arseven, O., Ceballos, C.J., Vives, V.I., Ramirez, R.C., Gomez, V.V., Medeiros-Neto, G., Kopp, P. Eur. J. Endocrinol. (2001) [Pubmed]
  18. The role of Math1 in inner ear development: Uncoupling the establishment of the sensory primordium from hair cell fate determination. Chen, P., Johnson, J.E., Zoghbi, H.Y., Segil, N. Development (2002) [Pubmed]
  19. The Notch ligands DLL1 and JAG2 act synergistically to regulate hair cell development in the mammalian inner ear. Kiernan, A.E., Cordes, R., Kopan, R., Gossler, A., Gridley, T. Development (2005) [Pubmed]
  20. Thyroid hormone affects Schwann cell and oligodendrocyte gene expression at the glial transition zone of the VIIIth nerve prior to cochlea function. Knipper, M., Bandtlow, C., Gestwa, L., Köpschall, I., Rohbock, K., Wiechers, B., Zenner, H.P., Zimmermann, U. Development (1998) [Pubmed]
  21. The role of Six1 in mammalian auditory system development. Zheng, W., Huang, L., Wei, Z.B., Silvius, D., Tang, B., Xu, P.X. Development (2003) [Pubmed]
  22. Protection against cisplatin ototoxicity by adenosine agonists. Whitworth, C.A., Ramkumar, V., Jones, B., Tsukasaki, N., Rybak, L.P. Biochem. Pharmacol. (2004) [Pubmed]
  23. Identification of Vangl2 and Scrb1 as planar polarity genes in mammals. Montcouquiol, M., Rachel, R.A., Lanford, P.J., Copeland, N.G., Jenkins, N.A., Kelley, M.W. Nature (2003) [Pubmed]
  24. Prestin, a new type of motor protein. Dallos, P., Fakler, B. Nat. Rev. Mol. Cell Biol. (2002) [Pubmed]
  25. A deafness mutation isolates a second role for the tectorial membrane in hearing. Legan, P.K., Lukashkina, V.A., Goodyear, R.J., Lukashkin, A.N., Verhoeven, K., Van Camp, G., Russell, I.J., Richardson, G.P. Nat. Neurosci. (2005) [Pubmed]
  26. Radixin deficiency causes deafness associated with progressive degeneration of cochlear stereocilia. Kitajiri, S., Fukumoto, K., Hata, M., Sasaki, H., Katsuno, T., Nakagawa, T., Ito, J., Tsukita, S., Tsukita, S. J. Cell Biol. (2004) [Pubmed]
  27. The site of action of neuronal acidic fibroblast growth factor is the organ of Corti of the rat cochlea. Pirvola, U., Cao, Y., Oellig, C., Suoqiang, Z., Pettersson, R.F., Ylikoski, J. Proc. Natl. Acad. Sci. U.S.A. (1995) [Pubmed]
  28. Hearing loss and retarded cochlear development in mice lacking type 2 iodothyronine deiodinase. Ng, L., Goodyear, R.J., Woods, C.A., Schneider, M.J., Diamond, E., Richardson, G.P., Kelley, M.W., Germain, D.L., Galton, V.A., Forrest, D. Proc. Natl. Acad. Sci. U.S.A. (2004) [Pubmed]
  29. Brain-type prostaglandin D synthetase occurs in the rat cochlea. Tachibana, M., Fex, J., Urade, Y., Hayaishi, O. Proc. Natl. Acad. Sci. U.S.A. (1987) [Pubmed]
  30. Mechanical responses of the mammalian cochlea. Ulfendahl, M. Prog. Neurobiol. (1997) [Pubmed]
  31. Cell cycle progression in gentamicin-damaged avian cochleas. Bhave, S.A., Stone, J.S., Rubel, E.W., Coltrera, M.D. J. Neurosci. (1995) [Pubmed]
  32. Specification of the mammalian cochlea is dependent on Sonic hedgehog. Riccomagno, M.M., Martinu, L., Mulheisen, M., Wu, D.K., Epstein, D.J. Genes Dev. (2002) [Pubmed]
  33. Activity-dependent transcription regulation of PSD-95 by neuregulin-1 and Eos. Bao, J., Lin, H., Ouyang, Y., Lei, D., Osman, A., Kim, T.W., Mei, L., Dai, P., Ohlemiller, K.K., Ambron, R.T. Nat. Neurosci. (2004) [Pubmed]
  34. Usher syndrome 1D and nonsyndromic autosomal recessive deafness DFNB12 are caused by allelic mutations of the novel cadherin-like gene CDH23. Bork, J.M., Peters, L.M., Riazuddin, S., Bernstein, S.L., Ahmed, Z.M., Ness, S.L., Polomeno, R., Ramesh, A., Schloss, M., Srisailpathy, C.R., Wayne, S., Bellman, S., Desmukh, D., Ahmed, Z., Khan, S.N., Kaloustian, V.M., Li, X.C., Lalwani, A., Riazuddin, S., Bitner-Glindzicz, M., Nance, W.E., Liu, X.Z., Wistow, G., Smith, R.J., Griffith, A.J., Wilcox, E.R., Friedman, T.B., Morell, R.J. Am. J. Hum. Genet. (2001) [Pubmed]
  35. Human nonsyndromic hereditary deafness DFNA17 is due to a mutation in nonmuscle myosin MYH9. Lalwani, A.K., Goldstein, J.A., Kelley, M.J., Luxford, W., Castelein, C.M., Mhatre, A.N. Am. J. Hum. Genet. (2000) [Pubmed]
  36. Expression of prestin-homologous solute carrier (SLC26) in auditory organs of nonmammalian vertebrates and insects. Weber, T., Gopfert, M.C., Winter, H., Zimmermann, U., Kohler, H., Meier, A., Hendrich, O., Rohbock, K., Robert, D., Knipper, M. Proc. Natl. Acad. Sci. U.S.A. (2003) [Pubmed]
  37. Hearing and hair cells are protected by adenoviral gene therapy with TGF-beta1 and GDNF. Kawamoto, K., Yagi, M., Stöver, T., Kanzaki, S., Raphael, Y. Mol. Ther. (2003) [Pubmed]
  38. Ionic mechanism of the efferent olivo-cochlear inhibition studied by cochlear perfusion in the cat. Desmedt, J.E., Robertson, D. J. Physiol. (Lond.) (1975) [Pubmed]
  39. Dopaminergic lateral efferent innervation of the guinea-pig cochlea: immunoelectron microscopy of catecholamine-synthesizing enzymes and effect of 6-hydroxydopamine. Eybalin, M., Charachon, G., Renard, N. Neuroscience (1993) [Pubmed]
  40. Expression of alpha and beta parvalbumin is differentially regulated in the rat organ of corti during development. Yang, D., Thalmann, I., Thalmann, R., Simmons, D.D. J. Neurobiol. (2004) [Pubmed]
  41. Molecular characterization and expression of maternally expressed gene 3 (Meg3/Gtl2) RNA in the mouse inner ear. Manji, S.S., Sørensen, B.S., Klockars, T., Lam, T., Hutchison, W., Dahl, H.H. J. Neurosci. Res. (2006) [Pubmed]
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