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Anatomical & Functional Abnormalities of the Horizontal Semicircular Canals in SLC26A4-Knockout Mice
Dept. of Otorhinolaryngology-Head and Neck Surgery,1,Seoul National Univ., Seoul2,Seoul National Univ. Bundang Hosp., Seongnam3,Eulji Univ., Eulji Medical Center, Seoul4,Gachon Univ. of Medicine and Science, Incheon5,Seoul National Univ. Hosp., Seoul6,Sensory Organ Research Institute, Seoul Natl Univ. Medical Research Center, Seoul6,Korea
Yong-Hwi AN, Byung Yoon CHOI12, Yong-Hwi AN13, Hyun Joon SHIM3, Jiyeon LEE4, Min Young KIM2, Jae-Jin SONG12, Seung Ha OH156, Jun Ho LEE 156, Ja-Won KOO126, Gyu Cheol HAN4
¸ñÀû: Pendrin, encoded by SLC26A4 gene, is a carrier protein expressed in inner ear epithelial cells that secrete HCO3 into endolymph. Details of vestibular ocular reflex (VOR) and anatomical abnormality of the lateral semicircular canal have not been characterized. Here we report the quantitative VOR responses and vestibular anatomy in pendrin deficient mice strain. ¹æ¹ý:Total 40 mice of wild type (Slc26a4+/+, N=9), Slc26a4 heterozygous knockout (Slc26a4¥Ä/+, N=10), transgenic mice with doxycycline- inducible partial expression of Slc26a4 (Tg(+)Slc26a4¥Ä/¥Ä, N=13) and Slc26a4 homozygous knockout (Slc26a4¥Ä/¥Ä, N=8) are used. Pendrin deficient mice were evaluated by auditory brainstem response (ABR), VOR, histology of the horizontal canal. Rotation test is performed more than 10 cycles with the maximal angular velocity of and 100 degree/sec in 0.1, 0.2, 0.5 Hz. °á°ú:Mean ABR thresholds of Slc26a4¥Ä/¥Ä mice were significantly elevated compared to Tg(+)Slc26a4¥Ä/¥Ä, Slc26a4¥Ä/+ and Slc26a4+/+ mice. In VOR testing, Slc26a4¥Ä/¥Ä mice had minimal response to any of the stimuli tested, whereas Tg(+)Slc26a4¥Ä/¥Ä and Slc26a4¥Ä/+ had an intermediate response compared to Slc26a4+/+. The mean gains of horizontal rotation at 0.1 Hz were 0.03 0.02 in Slc26a4¥Ä/¥Ä, 0.57 0.03 in Tg(+)Slc26a4¥Ä/¥Ä, 0.75 0.02 in Slc26a4¥Ä/+ and 0.87 0.03 in Slc26a4+/+mice, showing pendrin-dose dependent formation of VOR. A substantial portion of vestibular hair cells in the lateral canal from Slc26a4¥Ä/¥Ä are replaced by vacuoles, while there is no apparent difference between Slc26a4+/+ and Slc26a4¥Ä /+. °á·Ð:Vestibular function through VOR of horizontal canal is manifested with a pendrin dose-dependent pattern, while auditory function shows all-or-none phenotype in pendrin deficient mice. Mutations of SLC26A4 gene induce anatomical and functional deficits of vestibular hair cells of lateral semicircular canals.


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