The expression of a combined distal renal tubular acidosis(dRTA)-deafness related gene ATP6V0A4 in developing inner ears of the mice
LIU Yaqing
LI Qi
XIN Feng
YUAN Yongyi
Abstract：Objective Autosomal recessive distal renal tubular acidosis (dRTA), a severe disorder of acid–base homeostasis, is often accompanied by sensorineural deafness. The ATP6V0A4 is considered as the causative gene;how-ever, it is unknown how this gene causes the diseases. This study aims to investigate the expression of ATP6V0A4 in the mice inner ear at different stages of development. Methods Kunming mice at different developmental stages (Embryo 18, Postnatal 1, Postnatal 7, Postnatal 14, Postnatal 21) were used. The expression of Atp6v0a4 in the mice inner ear was analyzed using combined techniques of immunohistochemistry and immunofluorescence under confocal laser scan-ning microscopy. In addition, western-blot was used to analyze the expression of Atp6v0a4 quantitatively and qualita-tively in mice inner ear. Results Atp6v0a4 was steadily expressed in the inner and outer hair cells, stria vascularis, spi-ral ganglion, and endolymphatic sac during the inner ear development, as shown by confocal laser scanning microscopy. The western-blot showed that the expression of Atp6v0a4 is relatively stable in the organ of Corti, spiral ganglion, andstria vascularis, while the expression of Atp6v0a4 in vestibule decreased during inner ear development. Conclusions Ex-pression and localization of Atp6v0a4 in the mice inner ear at different developmental stages are demonstrated. This study may pave the way for further investigation in the Atp6v0a4 related deafness mechanisms.
Keywords：Atp6v0a4Expression and localizationInner ear
Publication Date：2017-01-01
Online Publishing Date：2025-08-15(First online date of this platform, not the publication date of the document)
Pages：6( 88-93 )