Abstract
GM1-gang are glycolipid storage diseases characterized by an accumulation of GM1-ganglioside and o deficiency of β-gal activity. Antibodies evoked against purified A1 isozyme of human liver were used to develop a sensitive radial immunodiffusion assay to quantitate the CRM of biological samples independent of their enzymic activity. β-Gal activities were determined in fibroblasts from controls and from patients with types 1 and 2 GM1-gang and Krabbe's disease using 4 mu-β-D-galactoside, (3H) lactosylceramide (assay 1 for “Krabbe's” enzyme, assay II for “GM1” enzyme), and (3H) GH1-ganglioside. The CRM was determined and the ratio of GM1, activity to CRM (CRM specific activity) was calculated.
In controls, the CRM was 0.31-0.43 μg/mg protein with a CRM specific activity of 512-588 nm/hr/μg. CRM of Krabbe fibroblasts and the β-gal activities were higher than those of controls resulting in CRM specific activity similar to controls. In GM1-gang, type 1, normal quantities of CRM were found with about a 500-fold decrease in the CRM specific activity. Almost twice the normal quantities of CRM were found in GM1-gang type 2 but the CRM specific activity was decreased only 100-fold. This assay provides new data regarding the molecular defect in GM1-gang, types 1 and 2 and may serve as a tool for the study of other defects of β-gal.
Article PDF
Author information
Authors and Affiliations
Rights and permissions
About this article
Cite this article
Ben-Yoseph, Y., Burton, B. & Nadlar, H. QUANTITATION OF GM1-β-GALACTOSIOASE (β-gal) CROSS REACTING MATERIAL (CRM) IN GM1-GANGLIOSIDOSES (GM1-gang). Pediatr Res 11, 452 (1977). https://doi.org/10.1203/00006450-197704000-00495
Issue Date:
DOI: https://doi.org/10.1203/00006450-197704000-00495