MK6-83

MK6-83 is an activator of the transient receptor potential cation channel, mucolipin subfamily (TRPML1). TRPML1 is mutated in mucolipidosis type IV (MLIV), a hereditary lysosomal storage disorder characterized by severe delays in intellectual and motor development. MK6-83 increases activity of TRPML1 in lysosomes expressing either wild type or F465L-mutated channels (EC50s = 110 and 100 nM, respectively) and in MLIV patient-derived fibroblasts. It is more effective for F465L mutations than F408Delta (EC50 = 1,230 nM). It also improves zinc trafficking out of lysosomes in F408Delta-expressing fibroblast cells.Formal Name: 5-methyl-N-[2-(1-piperidinyl)phenyl]-2-thiophenesulfonamide. CAS Number: 1062271-24-2. Molecular Formula: C16H20N2O2S2. Formula Weight: 336.5. Purity: >98%. Formulation: (Request formulation change), A crystalline solid. Solubility: DMF: 30 mg/mL, DMSO: 30 mg/mL, DMSO:PBS (pH 7.2) (1:2): 0.33 mg/mL, Ethanol: 5 mg/mL. SMILES: O=S(NC1=CC=CC=C1N2CCCCC2)(C3=CC=C(C)S3)=O. InChi Code: InChI=1S/C16H20N2O2S2/c1-13-9-10-16(21-13)22(19,20)17-14-7-3-4-8-15(14)18-11-5-2-6-12-18/h3-4,7-10,17H,2,5-6,11-12H2,1H3. InChi Key: IRGYSXZCDAWOOC-UHFFFAOYSA-N.