Kinetic properties of beta-galactosidase in Morquio B disease.
Some kinetic Properties of beta-galactosidase in crude extracts of cultured fibroblasts obtained from patients with Morquio B disease, GM1-gangliosidosis and from the controls were compared. The main defect in Morquio B seems to be the lowering of enzyme affinity to the substrates, while in the case of GM1-gangliosidosis of infantile type 1 a dramatic decrease of Vmax value concomitant with normal KM value was observed. Based on these observations possible interpretations of the clinical picture are discussed.
D005347:Fibroblasts / Q000201:enzymology; D005696:Galactosidases / Q000378:metabolism*; D005733:Gangliosidoses / Q000201:enzymology; D006801:Humans; D006863:Hydrogen-Ion Concentration; D007700:Kinetics; D009083:Mucopolysaccharidoses / Q000201:enzymology*; D001616:beta-Galactosidase / Q000037:antagonists & inhibitors / Q000378:metabolism*
D005696:Galactosidases; D001616:beta-Galactosidase