BACKGROUND: Otoconia are bio-crystals that couple mechanic forces to the sensory hair cells in the utricle and saccule, a process essential for us to sense linear acceleration and gravity for the purpose of maintaining bodily balance. In fish, structurally similar bio-crystals called otoliths mediate both balance and hearing. Otoconia abnormalities are common and can cause vertigo and imbalance in humans. However, the molecular etiology of these illnesses is unknown, as investigators have only begun to identify genes important for otoconia formation in recent years. RESULTS: To date, in-depth studies of selected mouse otoconial proteins have been performed, and about 75 zebrafish genes have been identified to be important for otolith development. CONCLUSIONS: This review will summarize recent findings as well as compare otoconia and otolith development. It will provide an updated brief review of otoconial proteins along with an overview of the cells and cellular processes involved. While continued efforts are needed to thoroughly understand the molecular mechanisms underlying otoconia and otolith development, it is clear that the process involves a series of temporally and spatially specific events that are tightly coordinated by numerous proteins. Such knowledge will serve as the foundation to uncover the molecular causes of human otoconia-related disorders.
BACKGROUND: Otoconia are bio-crystals that couple mechanic forces to the sensory hair cells in the utricle and saccule, a process essential for us to sense linear acceleration and gravity for the purpose of maintaining bodily balance. In fish, structurally similar bio-crystals called otoliths mediate both balance and hearing. Otoconia abnormalities are common and can cause vertigo and imbalance in humans. However, the molecular etiology of these illnesses is unknown, as investigators have only begun to identify genes important for otoconia formation in recent years. RESULTS: To date, in-depth studies of selected mouse otoconial proteins have been performed, and about 75 zebrafish genes have been identified to be important for otolith development. CONCLUSIONS: This review will summarize recent findings as well as compare otoconia and otolith development. It will provide an updated brief review of otoconial proteins along with an overview of the cells and cellular processes involved. While continued efforts are needed to thoroughly understand the molecular mechanisms underlying otoconia and otolith development, it is clear that the process involves a series of temporally and spatially specific events that are tightly coordinated by numerous proteins. Such knowledge will serve as the foundation to uncover the molecular causes of human otoconia-related disorders.
Authors: Louise S Bicknell; Sarah Walker; Anna Klingseisen; Tom Stiff; Andrea Leitch; Claudia Kerzendorfer; Carol-Anne Martin; Patricia Yeyati; Nouriya Al Sanna; Michael Bober; Diana Johnson; Carol Wise; Andrew P Jackson; Mark O'Driscoll; Penny A Jeggo Journal: Nat Genet Date: 2011-02-27 Impact factor: 38.330
Authors: H A Pedrozo; Z Schwartz; D D Dean; J L Harrison; J W Campbell; M L Wiederhold; B D Boyan Journal: Calcif Tissue Int Date: 1997-09 Impact factor: 4.333
Authors: Yoko Nakano; Chantal M Longo-Guess; David E Bergstrom; William M Nauseef; Sherri M Jones; Botond Bánfi Journal: J Clin Invest Date: 2008-03 Impact factor: 14.808