TRPML

TRPML is a family of transient receptor potential ion channels.

The three members of the TRPML sub-family are not well characterized. TRPML1 is known to be localized in late endosomes. This subunit also contains a lipase domain between its S1 and S2 segments. While the function of this domain is unknown it has been proposed that it is involved in channel regulation. Physiological studies have described TRPML1 channels as proton leak channels in lysosomes responsible for preventing these organelles from becoming too acidic. TRPML2 and TRPML3 have yet to be characterized.

Deficiencies can lead to enlarged vacuoles.

Genes

 * (TRPML1), (TRPML2),  (TRPML3)