TRPML (transient receptor potential cation channel, mucolipin subfamily) comprises a group of three evolutionarily related proteins that belongs to the large family of
transient receptor potential ion channel
Ion channels are pore-forming membrane proteins that allow ions to pass through the channel pore. Their functions include establishing a resting membrane potential, shaping action potentials and other electrical signals by Gating (electrophysiol ...
s. The three proteins
TRPML1,
TRPML2 and
TRPML3 are encoded by the mucolipin-1 (''
MCOLN1''), mucolipin-2 (''
MCOLN2'') and mucolipin-3 (''
MCOLN3'') genes, respectively.
The three members of the TRPML ("ML" for mucolipin) sub-family are not extremely well characterized.
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
A proton is a stable subatomic particle, symbol , Hydron (chemistry), H+, or 1H+ with a positive electric charge of +1 ''e'' (elementary charge). Its mass is slightly less than the mass of a neutron and approximately times the mass of an e ...
leak channels in
lysosome
A lysosome () is a membrane-bound organelle that is found in all mammalian cells, with the exception of red blood cells (erythrocytes). There are normally hundreds of lysosomes in the cytosol, where they function as the cell’s degradation cent ...
s responsible for preventing these organelles from becoming too acidic.
and
TRPML3 more poorly characterized than
TRPML1.
Deficiencies can lead to enlarged vesicles.
Genes
* (
TRPML1)
* (
TRPML2)
* (
TRPML3)
References
External links
*
*
{{Transient receptor potential channel modulators
Membrane proteins
Ion channels