Article
Synthesis and use of novel fluorescent glycosphingolipids for estimating beta-glucosidase activity in vitro in the absence of detergents and subtyping Gaucher disease variants following administration into intact cells.
Biochimica et biophysica acta - 29 Sept 1993
Agmon V, Cherbu S, Dagan A, Grace M, Grabowski G A, Gatt S
Abstract excerpt
Two novel fluorescent glycolipids, LRO-glucosylceramide (LRO-GC) and LRO-trihexosylceramide (LRO-THC) were synthesized and utilized for estimating activities of the lysosomal, acid beta-glucosidase in cell extracts and intact skin fibroblasts, derived from normal individuals and patients with Gau...
Topics
- Cells, Cultured
- Detergents
- Fluorescent Dyes
- Gaucher Disease
- Genotype
- Glucosylceramides
- Glycosphingolipids
- Humans
- Point Mutation
- Rhodamines
- Skin
- Trihexosylceramides