機能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.
組織特異性Kidney. Moderately expressed in aortic vascular smooth muscle and endothelial cells, and at a slightly higher level in the coronary vascular smooth muscle.
関連疾患Hypophosphatemic rickets, X-linked recessive Nephrolithiasis 2 Nephrolithiasis 1 Low molecular weight proteinuria with hypercalciuria and nephrocalcinosis
配列類似性Belongs to the chloride channel (TC 2.A.49) family. ClC-5/CLCN5 subfamily. Contains 2 CBS domains.
翻訳後修飾Ubiquitinated by NEDD4L in the presence of albumin; which promotes endocytosis and proteasomal degradation.