Current methods to analyze lysosome morphology, positioning, motility and function

DC Barral, L Staiano, C Guimas Almeida, DF Cutler… - Traffic, 2022 - Wiley Online Library
Since the discovery of lysosomes more than 70 years ago, much has been learned about
the functions of these organelles. Lysosomes were regarded as exclusively degradative …

Plant glycosides and glycosidases: a treasure-trove for therapeutics

K Kytidou, M Artola, HS Overkleeft… - Frontiers in plant …, 2020 - frontiersin.org
Plants contain numerous glycoconjugates that are metabolized by specific
glucosyltransferases and hydrolyzed by specific glycosidases, some also catalyzing …

The human disease gene LYSET is essential for lysosomal enzyme transport and viral infection

CM Richards, S Jabs, W Qiao, LD Varanese… - Science, 2022 - science.org
Lysosomes are key degradative compartments of the cell. Transport to lysosomes relies on
GlcNAc-1-phosphotransferase–mediated tagging of soluble enzymes with mannose 6 …

Neuronal soma-derived degradative lysosomes are continuously delivered to distal axons to maintain local degradation capacity

T Farfel-Becker, JC Roney, XT Cheng, S Li, SR Cuddy… - Cell reports, 2019 - cell.com
Neurons face the challenge of maintaining cellular homeostasis through lysosomal
degradation. While enzymatically active degradative lysosomes are enriched in the soma …

Activity-based protein profiling: Recent advances in medicinal chemistry

H Deng, Q Lei, Y Wu, Y He, W Li - European journal of medicinal chemistry, 2020 - Elsevier
Activity-based protein profiling (ABPP) has become an emerging chemical proteomic
approach to illustrate the interaction mechanisms between compounds and proteins. This …

Rational Design of Mechanism-Based Inhibitors and Activity-Based Probes for the Identification of Retaining α-l-Arabinofuranosidases

NGS McGregor, M Artola, A Nin-Hill… - Journal of the …, 2020 - ACS Publications
Identifying and characterizing the enzymes responsible for an observed activity within a
complex eukaryotic catabolic system remains one of the most significant challenges in the …

Glucocerebrosidase: functions in and beyond the lysosome

DEC Boer, J van Smeden, JA Bouwstra… - Journal of clinical …, 2020 - mdpi.com
Glucocerebrosidase (GCase) is a retaining β-glucosidase with acid pH optimum
metabolizing the glycosphingolipid glucosylceramide (GlcCer) to ceramide and glucose …

Animal Models for the Study of Gaucher Disease

O Cabasso, A Kuppuramalingam, L Lelieveld… - International Journal of …, 2023 - mdpi.com
In Gaucher disease (GD), a relatively common sphingolipidosis, the mutant lysosomal
enzyme acid β-glucocerebrosidase (GCase), encoded by the GBA1 gene, fails to properly …

Selective labelling of GBA2 in cells with fluorescent β-d-arabinofuranosyl cyclitol aziridines

Q Su, M Louwerse, RF Lammers, E Maurits… - Chemical …, 2024 - pubs.rsc.org
GBA2, the non-lysosomal β-glucosylceramidase, is an enzyme involved in glucosylceramide
metabolism. Pharmacological inhibition of GBA2 by N-alkyl iminosugars is well tolerated …

Functionalized cyclophellitols are selective glucocerebrosidase inhibitors and induce a bona fide neuropathic Gaucher model in zebrafish

M Artola, CL Kuo, LT Lelieveld… - Journal of the …, 2019 - ACS Publications
Gaucher disease is caused by inherited deficiency in glucocerebrosidase (GBA, a retaining
β-glucosidase), and deficiency in GBA constitutes the largest known genetic risk factor for …