Document Detail


Decreased amiloride-sensitive Na+ absorption in collecting duct principal cells isolated from BPK ARPKD mice.
MedLine Citation:
PMID:  14559716     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
The main feature of polycystic kidney diseases (PKD) is formation and progressive enlargement of renal cysts. Alterations in epithelial cell proliferation, extracellular matrix, and ion transport are thought to contribute to cyst enlargement and loss of renal function. Abnormal Cl- secretion is implicated in cyst enlargement in autosomal dominant PKD (ADPKD), but little is known about transport abnormalities in autosomal recessive PKD (ARPKD). We developed a method to isolate collecting duct (CD) principal cells (site of the lesion in ARPKD) from normal and ARPKD mice. A transgenic mouse (Hoxb7/GFP) in which enhanced green fluorescent protein (GFP) is expressed in CDs was bred with an ARPKD mouse (BPK), and GFP-positive cells from normal and cystic mice were selected by fluorescence-activated cell sorting. GFP-positive CD cells (>95 +/- 3%) obtained from either normal or cystic mice formed high-resistance, polarized epithelial monolayers. Expression patterns for marker proteins and the presence of a central cilium confirmed that the monolayers are composed of principal cells. Under basal conditions, the Cl- secretory responses elicited by elevation of cAMP or calcium were not significantly different between normal and cystic monolayers. In contrast, the amiloride-sensitive short-circuit current was significantly reduced in monolayers of cells isolated from cystic mice (12.9 +/- 1.6 microA/cm2; n = 10) compared with monolayers of cells isolated from normal mice (27.3 +/- 3.4 microA/cm2; n = 12). The results of these studies suggest that epithelial sodium channel-mediated sodium absorption is decreased in principal cells of ARPKD CD cysts and that the reduction in sodium absorption may contribute to the accumulation of luminal fluid.
Authors:
Elias I Veizis; Cathleen R Carlin; Calvin U Cotton
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Publication Detail:
Type:  Journal Article; Research Support, Non-U.S. Gov't; Research Support, U.S. Gov't, P.H.S.     Date:  2003-10-14
Journal Detail:
Title:  American journal of physiology. Renal physiology     Volume:  286     ISSN:  1931-857X     ISO Abbreviation:  Am. J. Physiol. Renal Physiol.     Publication Date:  2004 Feb 
Date Detail:
Created Date:  2004-01-06     Completed Date:  2004-02-24     Revised Date:  2011-04-28    
Medline Journal Info:
Nlm Unique ID:  100901990     Medline TA:  Am J Physiol Renal Physiol     Country:  United States    
Other Details:
Languages:  eng     Pagination:  F244-54     Citation Subset:  IM    
Affiliation:
Department of Pediatrics, Rainbow Center for Childhood PKD, Case Western Reserve University, Cleveland, OH 44106-4948, USA.
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MeSH Terms
Descriptor/Qualifier:
Animals
Cells, Cultured
Chlorides / metabolism
Electric Conductivity
Epithelial Sodium Channel
Flow Cytometry
Green Fluorescent Proteins
Indicators and Reagents / metabolism
Kidney Tubules, Collecting / cytology*,  metabolism*
Luminescent Proteins / genetics
Mice
Mice, Inbred BALB C
Mice, Transgenic
Polycystic Kidney, Autosomal Recessive / metabolism*,  physiopathology
Sodium / metabolism
Sodium Channels / metabolism*
Grant Support
ID/Acronym/Agency:
P30-DK-27651/DK/NIDDK NIH HHS; P50-DK-57306/DK/NIDDK NIH HHS
Chemical
Reg. No./Substance:
0/Chlorides; 0/Epithelial Sodium Channel; 0/Indicators and Reagents; 0/Luminescent Proteins; 0/Sodium Channels; 147336-22-9/Green Fluorescent Proteins; 7440-23-5/Sodium

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