4-Methylumbelliferyl 6-thio-Palmitate-β-D-Glucopyranoside

4-Methylumbelliferyl 6-thio-palmitate-β-D-glucopyranoside is a fluorogenic substrate of palmitoyl-protein thioesterase (PPT, also known as CLN1), a lysosomal hydrolase that removes long-chain fatty acyl groups from modified cysteine residues in proteins. 4-Methylumbelliferyl 6-thio-palmitate-β-D-glucopyranoside is cleaved by PPT/CLN1 to release the fluorescent moiety 4-methylumbelliferyl (4-MU). 4-MU fluorescence is pH-dependent with excitation maxima of 320 and 360 nm at low (1.97-6.72) and high (7.12-10.3) pH, respectively, and an emission maximum ranging from 445 to 455 nM, increasing as pH decreases.¹ This substrate is used in assays that measure PPT activity, which is commonly deficient in the neurodegenerative disorder known as infantile neuronal ceroid lipofuscinosis.^2,3

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1. Zhi, H., Wang, J., Wang, S., et al. Fluorescent properties of hymecromone and fluorimetric analysis of hymecromone in compound dantong capsule. J. Spectrosc. 147128 (2013).

2. Das, A.K., Lu, J.Y., and Hofmann, S.L. Biochemical analysis of mutations in palmitoyl-protein thioesterase causing infantile and late-onset forms of neuronal ceroid lipofuscinosis. Hum. Mol. Genet. 10(13), 1431-1439 (2001).

3. Vozni, Y.V., Keulemans, J.L.M., Mancini, G.M.S., et al. A new simple enzyme assay for pre- and postnatal diagnosis of infantile neuronal ceroid lipofuscinosis (INCL) and its variants. J. Med. Genet. 36(6), 471-474 (1999).

Brand, S., Roy, S., Schröder, P., et al. Combined proteomic and in silico target identification reveal a role for 5-lipoxygenase in developmental signaling pathways. Cell Chem. Biol. 25(9), 1095-1106 (2018).