Article
Intracellular degradation of sulforhodamine-GM1: use for a fluorescence-based characterization of GM2-gangliosidosis variants in fibroblasts and white blood cells.
Clinica chimica acta; international journal of clinical chemistry - 29 Mar 1996
Agmon V, Khosravi R, Marchesini S, Dinur T, Dagan A, Gatt S, Navon R
Abstract excerpt
A novel fluorescent ganglioside, sulforhodamine-GM1 was administered into cells derived from carriers and patients with different subtypes of GM2 gangliosidosis, resulting from various mutations in the gene encoding the lysosomal enzyme hexosaminidase (Hex) A. The cells used were skin fibroblasts...
Topics
- Cell Line
- Fibroblasts
- Fluorescence
- G(M1) Ganglioside
- Gangliosidoses
- Humans
- Leukocytes
- Mutation
- Rhodamines
- Tay-Sachs Disease