TRPML

The mucolipin receptor family (TRPML) is a member of the transient receptor potential (TRP) superfamily of ion channels. It consists of three mammalian members (TRPML1-3). Acidic pH appears to regulate the ion current properties of all three TRPML channels.

Products
Background
Literature
Gene Data

Activators

Cat No Product Name / Activity
5547 MK6-83
TRPML channel activator
4746 ML SA1
Activator of TRPML channels
4907 SN 2
Selective TRPML3 channel activator

Blockers

Cat No Product Name / Activity
4741 Gadolinium chloride
TRPML3 blocker; stretch-activated calcium channel blocker

The mucolipin receptor family (TRPML) is a member of the transient receptor potential (TRP) superfamily of ion channels. It consists of three mammalian members (TRPML1-3). Acidic pH appears to regulate the ion current properties of all three TRPML channels.

TRPML1 is a cation-selective ion channel that is important for sorting/transport of endosomes in the late endocytotic pathway and specifically fusion between late endosome-lysosome hybrid vesicles. Therefore they are thought to control delivery of cellular material to lysosomes. TRPML1 has also been implicated in lysosomal dysfunction, which is associated with increased autophagy. Mutations in the gene encoding the TRPML1 channel (MCOLN1) are the cause of the neurodegenerative disorder mucolipidosis type IV (MLIV) in humans. TRPML2 channels are thought to be localized to the cell surface and recycling endosomes. TRPML3 channels appear to play a role in the regulation of endocytosis and autophagy. A gain-of-function mutation in the TRPML3 channel causes the varitint-waddler phenotype, whereas overexpression of TRPML3 leads to reduced endocytosis and increased autophagy with recruitment of TRPML3 into autophagosomes.

All three channels have been described to be capable of colocalizing with each other, in a subset of intracellular vesicles. Recombinant as well as native TRPML channels have been shown to physically interact with each other in homo- and heteromultimeric combinations.

External sources of pharmacological information for TRPML :

Literature for TRPML

Autophagy

Autophagy Scientific Review

Written by Patricia Boya and Patrice Codogno, this review summarizes the molecular mechanisms, physiology and pathology of autophagy. The role of autophagy in cell death and its links to disease are also discussed. Compounds available from Tocris are listed.

Autophagy

Autophagy Poster

Autophagy is a cellular process used by cells for degradation and recycling. Written by Patricia Boya and Patrice Codogno, this poster summarizes the molecular machinery, physiology and pathology of autophagy. Compounds available from Tocris are listed.

TRPML Gene Data

Gene Species Gene Symbol Gene Accession No. Protein Accession No.
Mucolipin 1 Human MCOLN1 NM_020533 Q9GZU1
Mouse Mcoln1 NM_053177 NP_444407
Rat Mcoln1 NM_001105903 NP_001099373
Mucolipin 2 Human MCOLN2 NM_153259 Q8IZK6
Mouse Mcoln2 NM_026656 NP_080932
Rat Mcoln2 NM_001039005 NP_001034094
Mucolipin 3 Human MCOLN3 NM_018298 Q8TDD5
Mouse Mcoln3 NM_134160 NP_598921
Rat Mcoln3 NM_001012059 NP_001012059