ATP13A1
Description
The ATP13A1 (ATPase 13A1) is a protein-coding gene located on chromosome 19.
ATP13A1 is an endoplasmic reticulum (ER) translocase that removes mitochondrial transmembrane proteins that have been mistakenly targeted to the ER. It acts as a dislocase, extracting these mislocalized proteins from the ER membrane using ATP. ATP13A1 specifically binds to mitochondrial tail-anchored transmembrane proteins. Its substrate-binding pocket is unusually large, allowing it to recognize and bind moderately hydrophobic transmembrane regions with short hydrophilic domains.
ATP13A1 is also known as ATP13A, CGI-152.
