Enzyme-linked Immunosorbent Assay for the Ganglioside GM2-Activator Protein. Screening of Normal Human Tissues and Body Fluids, of Tissues of GM2Gangliosidosis, and for Its Subcellular Localization
- 1 January 1984
- journal article
- research article
- Published by Walter de Gruyter GmbH in Hoppe-Seyler´s Zeitschrift Für Physiologische Chemie
- Vol. 365 (1) , 347-356
- https://doi.org/10.1515/bchm2.1984.365.1.347
Abstract
An enzyme-linked immunosorbent assay was developed for the quantitation of the human activator protein for ganglioside GM2 degradation by .beta.-N-acetyl-D-hexosaminidase A. With this assay, various tissues and body fluids of normal subjects and of patients with variant forms of GM2 gangliosidosis were screened for the GM2-activator content. The highest content of this protein was found in kidney. Tissues from patients with variant AB of infantile GM2 gangliosidosis contained only low levels (up to 5% of that of normal controls) of cross-reacting material. On subcellular fractionation of normal human skin fibroblasts, the activator was found to codistribute with the lysosomal marker enzyme .beta.-hexosaminidase.This publication has 15 references indexed in Scilit:
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