Fibroblasts sialidase

Usuki, S., Hoops, P., and Sweeley, C. C. (1988). Growth control of human foreskin fibroblasts and inhibition of extracellular sialidase activity by 2-deoxy-2,3-dehydro-N-acetylneura-minic acid. /. Biol. Chem. 263, 10595-10599. 

As many conflicting observations have been made in this field, better characterization of human sialidases, especially those from the readily obtainable fibroblasts, is needed, as well as standardized, natural substrates and sialidase-assay conditions. 

Prepare a fresh fibroblast homogenate in 130 pi distilled water (note sialidase is highly labile upon sonification and freezing ), using a small (200-pl) Potter appara-... 

Table 4.3.6 Normal and pathological values of sialidase and f-galactosidase activity in cultured fibroblasts of controls and patients with sialidosis or galactosialidosis...

GalNAc-protein (sialidase-treated ovine submaxillary mucin) InefiFective with low molecular weight acceptors NAN- (2 6) -GalNAc-protein Submaxillary glands (pork, sheep, bovine) fibroblasts Section V, A... 

In a patient classified as mucolipidosis I, Ckmzetal (1977) and Spranger /. (1977) demonstrated a severe deficiency of an acid neuraminidase (sialidase N-acetyl neuraminic acid hydrolase, E.C. 3.2.1.18) in his cultured fibroblasts. The patient had a neurodegenerative disorder with myoclonus, skeletal changes like in Hurler disease, and cherry-red spots in the maculae of his eyes. In addition to the neuraminidase defect, the fibroblasts of the patient accumulated abnormal amounts of sialic acid-containing compounds. The patient excreted excessive quantities of sialyloligosaccharides in the urine (Michalski etal 1977). Fibroblasts from the parents of another such patient had activities of neuraminidase which were intermediate between patients and controls (Cantz and... 

Lieser, M., Harms, E., Kern, H., Bach, G., and Cantz, M., 1989, Ganglioside GM3 sialidase activity in fibroblasts of normal individuals and of patients with sialidosis and mucolipidosis IV, Biochem. J. 260 69-74. 

Schengrund, C.-L., Rosenberg, A., and Repman, M. A., 1976, Ecto-ganglioside-sialidase activity of herpes simpex virus transformed hamster embryo fibroblasts, J. Cell Biol. 70 555-561. 

Schneider-Jakob, H. R., and Cantz, M., 1991, Lysosomal and plasma membrane gangliosides GM3 sialidases of cultured human fibroblasts, Biol. Chem. Hoppe-Seyler 372 443-450. 

Yogeeswaran, G., and Hakomori, S., 1975, Cell contact-dependent ganglioside changes in mouse 3T3 fibroblasts and a suppressed sialidase activity on cell contact. Biochemistry 14 2151. 

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