FunctionProton-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.
Tissue specificityKidney. Moderately expressed in aortic vascular smooth muscle and endothelial cells, and at a slightly higher level in the coronary vascular smooth muscle.
Involvement in diseaseHypophosphatemic rickets, X-linked recessive Nephrolithiasis 2 Nephrolithiasis 1 Low molecular weight proteinuria with hypercalciuria and nephrocalcinosis
Sequence similaritiesBelongs to the chloride channel (TC 2.A.49) family. ClC-5/CLCN5 subfamily. Contains 2 CBS domains.
Post-translational modificationsUbiquitinated by NEDD4L in the presence of albumin; which promotes endocytosis and proteasomal degradation.