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    Hoppe Seylers Z Physiol Chem. 1984 Mar;365(3):347-56.

    Enzyme-linked immunosorbent assay for the ganglioside GM2-activator protein. Screening of normal human tissues and body fluids, of tissues of GM2 gangliosidosis, and for its subcellular localization.

    Banerjee A, Burg J, Conzelmann E, Carroll M, Sandhoff K.

    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 their 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 co-distribute with the lysosomal marker enzyme beta-hexosaminidase.

    PMID: 6724528 [PubMed - indexed for MEDLINE]

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