Progress of the renal chloride channel CLC-5 and Dent disease
10.3760/cma.j.issn.1673-4408.2015.01.022
- VernacularTitle:肾脏氯离子通道CLC-5与Dent病的研究进展
- Author:
Zhenjuan LIU
- Publication Type:Journal Article
- Keywords:
CLC-5;
Voltage-gated chloride channel;
Megalin;
Dent disease
- From:
International Journal of Pediatrics
2015;42(1):73-75
- CountryChina
- Language:Chinese
-
Abstract:
Chloride channel CLC-5 is a voltage-dependent gated channel.The voltage-gated characteristic of CLC-5 is not only regulated by glutamate E211 and E268,but also by lysine K210.In proximal renal tubule,CLC-5 can interact with megalin protein,and adjust the reabsorption of albumin together.CLC-5 can also affect the activities of the sodium hydrogen exchanger isoform 3 in proximal renal tubule.CLCN5 gene mutations can lead to Dent disease,and recent studies have found some new pathogenic mutants of CLC-5,VS05G,L266V and G446A,and so on.CLCN5 can also mutate together with ORCL1,and then result in Dent disease.However,the regulatory mechanism of the voltage-gated channel,physiological functions and molecular mechanism,and Dent disease are still not entirely clear.In this paper,we will review these problems of CLC-5.
