The mucolipin-2 (TRPML2) ion channel: a tissue-specific protein crucial to normal cell function.

摘要: The discovery of the TRPML subfamily ion channels has created an exciting niche in fields membrane trafficking, signal transduction, autophagy, and metal homeostasis. protein consists three members, TRPML1, TRPML2, TRPML3, which are encoded by MCOLN1, MCOLN2, MCOLN3 genes, respectively. They non-selective cation with six predicted transmembrane domains intracellular amino- carboxyl-terminus regions. localize to plasma membrane, endosomes, lysosomes cells. TRPML1 is associated human lysosomal storage disease known as mucolipidosis type IV (MLIV), but TRPML2 TRPML3 have not been linked a disease. Although expressed many tissues, varied limited set while more expression pattern where it mostly detected lymphoid myeloid tissues. This review focuses on because appears play important, yet unrecognized role immune system. While evidence indirect, we present discuss relevant data that strengthen connection cellular immunity. We also functional redundancy between proteins, how such features could be exploited potential therapeutic strategy for MLIV may complement certain phenotypic alterations cells briefly examine challenges complementation. In conclusion, function still remains obscure, emerging show serve critical cell development inflammatory responses.