TABLE 26

TRPML1 channel

Channel name TRPML11,2
Description Nonselective cation channel subunit
Other names MCOLN1, mucolipin 1
Molecular information Human unigene: Hs0.310431, chr. 5q31
Mouse unigene: Mm0.226899, chr. 18 C
Associated subunits Not established
Functional assays Patch-clamp, calcium imaging
Current Not established
Conductance 300pS?
Ion selectivity PNa/PCa ∼1
Activation Not established
Inactivation Not established
Activators [Ca2+]i?
Gating inhibitors Not established
Blockers Amiloride (130 μM), Gd3+, La3+, Ni2+
Radioligands Not established
Channel distribution Widely expressed, heart kidney, testis, blood
Physiological functions Not established
Mutations and pathophysiology Not established
Comments Electrophysiological properties not firmly established
  • chr., chromosome.

  • 1. LaPlante JM, Falardeau J, Sun M, KanazirskaM, Brown EM, Slaugenhaupt SA, and Vassilev PM (2002) Identification and characterization of the single channel function of human mucolipin-1 implicated in mucolipidosis type IV, a disorder affecting the lysosomal pathway. FEBS Lett 532:183-187

  • 2. LaPlante JM, Ye CP, Quinn SJ, Goldin E, Brown EM, Slaugenhaupt SA, and Vassilev PM (2004) Functional links between mucolipin-1 and Ca2+-dependent membrane trafficking in mucolipidosis IV. Biochem Biophys Res Commun 322:1384-1391