acid-phosphatase and Leukodystrophy--Globoid-Cell

A histochemical study of the enzyme profile of Krabbe's cells revealed a high activity of lysosomal enzymes, including the tartrate-resistant isoenzyme of acid phosphatase, and considerable activity of extralysosomal nonspecific esterase and mitochondrial and extramitochondrial dehydrogenases. The presence of lysozyme was demonstrated. This generally high metabolic activity was found closely reminiscent of that in Gaucher's cells and in epitheloid transformed macrophages though quite unlike the exclusive predominance of lysosomal enzyme activity typical of classical foamy storage cells in Niemann-Pick and Wolman's disease.

Topics: Acid Phosphatase; Child, Preschool; Esterases; Humans; Leukodystrophy, Globoid Cell; Male; Muramidase; Oxidoreductases

Topics: Acid Phosphatase; Aspartate Aminotransferases; Brain; Chromatography; Diffuse Cerebral Sclerosis of Schilder; Glucosephosphate Dehydrogenase; Humans; Infant; Infant, Newborn; L-Lactate Dehydrogenase; Leukodystrophy, Globoid Cell; Lipids; Neurochemistry; Neuroglia; Phospholipids; Tuberous Sclerosis

Topics: Acid Phosphatase; Child; Diffuse Cerebral Sclerosis of Schilder; Electrons; Humans; L-Lactate Dehydrogenase; Leukodystrophy, Globoid Cell; Microscopy; Microscopy, Electron; Oxidoreductases; Tuberous Sclerosis