Otoancorin Explained
Otoancorin is a protein found in the vertebrate inner ear, on the sensory epithelia where it connects to the gel matrix.[1]
Otoancorin is found in the cochlea, utricule, saccule, and under the cupulae on the surface of apical dells in the sensory epithelia.[2]
In humans the gene that encodes otoancorin is called OTOA. It is on chromosome 16p12.2 and contains 28 exons. A recessive mutation in this gene called IVS12+2T>C results in deafness. The human protein has 1,153 amino acids.[2]
In the mouse, this protein has 1088 amino acids.[2] In mice otoancorin is needed to attach the tectorial membrane to the inner hair cells in the cochlea.[3]
Notes and References
- Deans MR, Peterson JM, Wong GW . Mammalian Otolin: a multimeric glycoprotein specific to the inner ear that interacts with otoconial matrix protein Otoconin-90 and Cerebellin-1 . PLOS ONE . 5 . 9 . e12765 . September 2010 . 20856818 . 2939893 . 10.1371/journal.pone.0012765 . 2010PLoSO...512765D . free .
- Zwaenepoel I, Mustapha M, Leibovici M, Verpy E, Goodyear R, Liu XZ, Nouaille S, Nance WE, Kanaan M, Avraham KB, Tekaia F, Loiselet J, Lathrop M, Richardson G, Petit C . 6 . Otoancorin, an inner ear protein restricted to the interface between the apical surface of sensory epithelia and their overlying acellular gels, is defective in autosomal recessive deafness DFNB22 . Proceedings of the National Academy of Sciences of the United States of America . 99 . 9 . 6240–5 . April 2002 . 11972037 . 122933 . 10.1073/pnas.082515999 . 2002PNAS...99.6240Z . free .
- Weddell T, Legan PK, Lukashkina VA, Goodyear RJ, Welstead L, Petit C, Russell IJ, Lukashkin AN, Richardson GP . 6 . 10.1063/1.3658074. Otoancorin Knockout Mice Reveal Inertia is the Force for Hearing. AIP Conference Proceedings. 2011 . American Institute of Physics Conference Series. 1403. 1. 139–140. 2011AIPC.1403..139W.