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Heterogeneity in the processing of CLCN5 mutants related to Dent disease.

Authors :
Grand, Teddy
L'Hoste, Sébastien
Mordasini, David
Defontaine, Nadia
Keck, Mathilde
Pennaforte, Thomas
Genete, Mathieu
Laghmani, Kamel
Teulon, Jacques
Lourdel, Stéphane
Source :
Human Mutation; Apr2011, Vol. 32 Issue 4, p476-483, 8p, 5 Diagrams, 1 Chart, 2 Graphs
Publication Year :
2011

Abstract

The article discusses a study on the consequences of missense mutations of the electrogenic Cl<superscript>-</superscript>/H<superscript>+</superscript> exchanger ClC-5 in Xenopus laevis oocytes and HEK293 cells to determine other mechanisms involved in Dent disease. The disease which is an X-linked recessive rental tubular disorder is often associated with CLCN5 gene mutations. Improper N-glycosylation was observed in three mutants. A novel mechanism resulting to ClC-5 dysfunction in Dent disease was identified.

Details

Language :
English
ISSN :
10597794
Volume :
32
Issue :
4
Database :
Complementary Index
Journal :
Human Mutation
Publication Type :
Academic Journal
Accession number :
59319949
Full Text :
https://doi.org/10.1002/humu.21467