Article
TRP-ML1 is a lysosomal monovalent cation channel that undergoes proteolytic cleavage.
The Journal of biological chemistry - 30 Dec 2005
Kiselyov Kirill, Chen Jin, Rbaibi Youssef, Oberdick Daniel, Tjon-Kon-Sang Sandra, Shcheynikov Nikolay, Muallem Shmuel, Soyombo Abigail
Abstract excerpt
Mutations in the gene MCOLN1 coding for the TRP (transient receptor potential) family ion channel TRP-ML1 lead to the lipid storage disorder mucolipidosis type IV (MLIV). The function and role of TRP-ML1 are not well understood. We report here that TRP-ML1 is a lysosomal monovalent cation channel. Both native and recombinant TRP-ML1 are cleaved resulting in two products. Recombinant TRP-ML1 is detected as the...
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