Quantitation of the Enzymically Deficient Cross Reacting Material in GM1 Gangliosidoses

Overview

Journal Am J Hum Genet

Publisher Cell Press

Specialty Genetics

Date 1977 Nov 1

PMID 412418

Citations 8

Authors

B K Burton

H L NADLER

Affiliations

Soon will be listed here.

Abstract

Normal quantities of GM1 beta-galactosidase cross reacting material (CRM) (0.31-0.47 microgram/mg protein) were detected by a sensitive radial immunodiffusion assay in skin fibroblasts from patients with GM1 gangliosidosis type 1 and adult variants, whereas elevated levels were found in GM1 gangliosidosis type 2 (0.41-0.72 microgram/mg protein). The specific activity of the immunologically CRM towards GM1 ganglioside of normal fibroblasts was about 500 times that of type 1, 100 times that of type 2, and 30 times that of the adult variants.

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