Histochemical demonstration of acid beta-D-galactosidase activity in cultured human skin fibroblasts and its application to single cell analyses.

Abstract

In 1970, Lojda reported the improved indigogenic method for β-D-galacto-sidase (β-gal). This method was applied to cultured skin fibroblasts from patients with three β-gal deficient disorders and from normal subjects. Cells from normal subjects showed the formation of insoluble blue indigo dye, whereas cells from the patients remained unstained. Clear-cut differences were observed in the intensity of the β-gal activities between cells from normal subjects and from the patients. This method was also applied to cultured amniotic fluid cells, and to the judgment of a genetic complementation study in restoration of β-gal activity.