Chemical Name: 5-Methyl-N-[2-(1-piperidinyl)phenyl]-2-thiophenesulfonamide
Biological ActivityMK6-83 is a TRPML channel activator (EC50 = 110 nM). Also activates F408Δ and F465L TRPML mutants (EC50 values are 0.1 and 1.23 μM respectively). Restores endolysosomal trafficking and zinc homeostasis in lysosomes of mucolipidosis type IV (MLIV) mutant fibroblasts.
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A small molecule restores function to TRPML1 mutant isoforms responsible for mucolipidosis type IV.
Chen et al.
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