8pej

Publication Abstract from PubMed

Acid beta-galactosidase (GLB1) and galactocerebrosidase (GALC) are retaining exo-beta-galactosidases involved in lysosomal glycoconjugate metabolism. Deficiency of GLB1 may result in the lysosomal storage disorders GM1 gangliosidosis, Morquio B syndrome, and galactosialidosis, and deficiency of GALC may result in Krabbe disease. Activity-based protein profiling (ABPP) is a powerful technique to assess the activity of retaining glycosidases in relation to health and disease. This work describes the use of fluorescent and biotin-carrying activity-based probes (ABPs) to assess the activity of both GLB1 and GALC in cell lysates, culture media, and tissue extracts. The reported ABPs, which complement the growing list of retaining glycosidase ABPs based on configurational isomers of cyclophellitol, should assist in fundamental and clinical research on various beta-galactosidases, whose inherited deficiencies cause debilitating lysosomal storage disorders.

The development of a broad-spectrum retaining beta-exo-galactosidase activity-based probe.,Kuo CL, Su Q, van den Nieuwendijk AMCH, Beenakker TJM, Offen WA, Willems LI, Boot RG, Sarris AJ, Marques ARA, Codee JDC, van der Marel GA, Florea BI, Davies GJ, Overkleeft HS, Aerts JMFG Org Biomol Chem. 2023 Sep 19. doi: 10.1039/d3ob01261a. PMID:37724332^[1]

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine.