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Molecular architecture underlying fluid absorption by the developing inner ear

 Keiji Honda  ;  Sung Huhn Kim  ;  Michael C Kelly  ;  Joseph C Burns  ;  Laura Constance  ;  Xiangming Li  ;  Fei Zhou  ;  Michael Hoa  ;  Matthew W Kelley  ;  Philine Wangemann  ;  Robert J Morell  ;  Andrew J Griffith 
 ELIFE, Vol.6 : e26851, 2017 
Journal Title
Issue Date
Animals ; Anion Transport Proteins / metabolism* ; Endolymph / metabolism* ; Endolymphatic Sac / embryology* ; Endolymphatic Sac / physiology* ; Gene Expression Profiling ; Mice ; Sodium Chloride / metabolism ; Sulfate Transporters
RNA-seq ; Slc26a4 ; developmental biology ; endolymphatic sac ; gene array ; mouse ; neuroscience ; organ culture ; stem cells
Mutations of SLC26A4 are a common cause of hearing loss associated with enlargement of the endolymphatic sac (EES). Slc26a4 expression in the developing mouse endolymphatic sac is required for acquisition of normal inner ear structure and function. Here, we show that the mouse endolymphatic sac absorbs fluid in an SLC26A4-dependent fashion. Fluid absorption was sensitive to ouabain and gadolinium but insensitive to benzamil, bafilomycin and S3226. Single-cell RNA-seq analysis of pre- and postnatal endolymphatic sacs demonstrates two types of differentiated cells. Early ribosome-rich cells (RRCs) have a transcriptomic signature suggesting expression and secretion of extracellular proteins, while mature RRCs express genes implicated in innate immunity. The transcriptomic signature of mitochondria-rich cells (MRCs) indicates that they mediate vectorial ion transport. We propose a molecular mechanism for resorption of NaCl by MRCs during development, and conclude that disruption of this mechanism is the root cause of hearing loss associated with EES.
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1. College of Medicine (의과대학) > Dept. of Otorhinolaryngology (이비인후과학교실) > 1. Journal Papers
Yonsei Authors
Kim, Sung Huhn(김성헌)
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