Document Detail


Deafness and renal tubular acidosis in mice lacking the K-Cl co-transporter Kcc4.
MedLine Citation:
PMID:  11976689     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
Hearing depends on a high K(+) concentration bathing the apical membranes of sensory hair cells. K(+) that has entered hair cells through apical mechanosensitive channels is transported to the stria vascularis for re-secretion into the scala media(). K(+) probably exits outer hair cells by KCNQ4 K(+) channels(), and is then transported by means of a gap junction system connecting supporting Deiters' cells and fibrocytes() back to the stria vascularis. We show here that mice lacking the K(+)/Cl(-) (K-Cl) co-transporter Kcc4 (coded for by Slc12a7) are deaf because their hair cells degenerate rapidly after the beginning of hearing. In the mature organ of Corti, Kcc4 is restricted to supporting cells of outer and inner hair cells. Our data suggest that Kcc4 is important for K(+) recycling() by siphoning K(+) ions after their exit from outer hair cells into supporting Deiters' cells, where K(+) enters the gap junction pathway. Similar to some human genetic syndromes(), deafness in Kcc4-deficient mice is associated with renal tubular acidosis. It probably results from an impairment of Cl(-) recycling across the basolateral membrane of acid-secreting alpha-intercalated cells of the distal nephron.
Authors:
Thomas Boettger; Christian A Hübner; Hannes Maier; Marco B Rust; Franz X Beck; Thomas J Jentsch
Related Documents :
11377879 - Ultrastructure of the inner ear of nkcc1-deficient mice.
7407849 - Light- and electron-microscopic analysis of a complex sensory organ: the tegula of locu...
1484699 - Cytodifferentiation within the developing human endolymphatic sac.
8151129 - Merkel cells of the terminal hair follicle of the adult human scalp.
2808149 - The nature and progression of injury in the organ of corti during ischemia.
11495929 - Evidence for an active process and a cochlear amplifier in nonmammals.
1511099 - Heterogeneity of cell populations that contribute to the formation of primordial follic...
9889919 - Are there significant gradients of po2 in cells?
7895279 - Regeneration and post-metamorphic development of the central nervous system in the prot...
Publication Detail:
Type:  Journal Article; Research Support, Non-U.S. Gov't    
Journal Detail:
Title:  Nature     Volume:  416     ISSN:  0028-0836     ISO Abbreviation:  Nature     Publication Date:  2002 Apr 
Date Detail:
Created Date:  2002-04-26     Completed Date:  2002-05-07     Revised Date:  2007-11-15    
Medline Journal Info:
Nlm Unique ID:  0410462     Medline TA:  Nature     Country:  England    
Other Details:
Languages:  eng     Pagination:  874-8     Citation Subset:  IM    
Affiliation:
Zentrum für Molekulare Neurobiologie, ZMNH, Universität Hamburg, Falkenried 94, 20246 Hamburg, Germany.
Export Citation:
APA/MLA Format     Download EndNote     Download BibTex
MeSH Terms
Descriptor/Qualifier:
Acidosis, Renal Tubular / genetics*,  pathology
Animals
Cochlea / metabolism,  pathology
Deafness / genetics*,  pathology
Hair Cells, Auditory / metabolism,  pathology
Hearing Tests
Humans
Ion Transport
Kidney / metabolism,  pathology
Mice
Mice, Inbred Strains
Potassium / metabolism
Symporters / deficiency*,  genetics*,  metabolism
Chemical
Reg. No./Substance:
0/SLC12A7 protein, human; 0/Slc12a7 protein, mouse; 0/Symporters; 7440-09-7/Potassium

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine


Previous Document:  AP-1 functions upstream of CREB to control synaptic plasticity in Drosophila.
Next Document:  Structural determinants for GoLoco-induced inhibition of nucleotide release by Galpha subunits.