Proton-coupled chloride transporter. Functions as antiport system and exchanges chloride ions against protons. Important for normal acidification of the endosome lumen. May play an important role in renal tubular function.
Kidney. Moderately expressed in aortic vascular smooth muscle and endothelial cells, and at a slightly higher level in the coronary vascular smooth muscle.
Involvement in disease
Hypophosphatemic rickets, X-linked recessive Nephrolithiasis 2 Nephrolithiasis 1 Low molecular weight proteinuria with hypercalciuria and nephrocalcinosis
Belongs to the chloride channel (TC 2.A.49) family. ClC-5/CLCN5 subfamily. Contains 2 CBS domains.
Ubiquitinated by NEDD4L in the presence of albumin; which promotes endocytosis and proteasomal degradation.