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.[1]
Deficiencies can lead to enlarged vacuoles.[2]
Genes
- MCOLN1 (TRPML1), MCOLN2 (TRPML2), MCOLN3 (TRPML3)
References
- ^ Nilius B, Owsianik G, Voets T, Peters JA (2007). "Transient receptor potential cation channels in disease". Physiol. Rev. 87 (1): 165-217. PMID 17237345.
- ^ Qian F, Noben-Trauth K (2005). "Cellular and molecular function of mucolipins (TRPML) and polycystin 2 (TRPP2)". Pflugers Arch. 451 (1): 277-85. doi:10.1007/s00424-005-1469-4. PMID 15971078.
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Membrane transport protein: ion channels |
| Ca2+: Calcium channel |
Voltage-dependent calcium channel (L-type/Cavα(1.1, 1.2, 1.3, 1.4), N-type, P-type/Cavα(2.1), Q-type, R-type, T-type, β-subunits (β1, β2, β4), γ-subunits (γ2) • Inositol triphosphate receptor • Ryanodine receptor • Cation channels of sperm • Two-pore channel |
| Na+: Sodium channel |
Navα (1.1, 1.2, 1.4, 1.5, 1.7, 1.9) • Navβ (1, 3, 4) • Epithelial sodium channel |
| K+: Potassium channel |
Voltage-gated (Kvα (1.1, 1.2, 1.3, 1.4, 1.5, 2.1, 4.2, 4.3, 7.1, 7.2, 7.3, 7.4, 10.1, 11.1/hERG) • Kvβ (1, 2), Shaker gene, KCNE1) • Calcium-activated (BK channel, SK channel, SK3) • Inward-rectifier Kir (1.1, 2.1, 2.2, 2.3, 3.1, 3.2, 3.4, 4.1, 4.2, 6.1, 6.2)) • Tandem pore domain K2P (1, 2, 3, 4, 6, 9) |
| Cl-: Chloride channel |
Cystic fibrosis transmembrane conductance regulator |
| Porin |
Aquaporin (1, 2, 3, 4) • Voltage-dependent anion channel (1) |
| Cations: TRP |
TRPA (1) • TRPC (1, 2, 3, 4, 4AP, 5, 6, 7) • TRPM (1, 2, 3, 4, 5, 6, 7, 8) • TRPML (Mucolipin-1) • TRPP (1, 2)• TRPV (1, 2, 3, 4, 5, 6) |
| Other/general |
Voltage-gated ion channel • Ligand-gated ion channel • Cyclic nucleotide-gated ion channel (α1, α3, β3, H1, H2, H4) • Stretch-activated ion channel |
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