| Popis: |
Galactosaemia is an inborn error of metabolism characterized by irreversible damage to neural tissue. To evaluate whether galactose metabolic disorders, (e.g. classical galactosaemia, galactokinase deficiency galactosaemia), is implicated for alterations of brain Mg2+-ATPase activity, various concentrations (1-16 mM) of galactose, galactose-1-phosphate, galactitol, glucose-1-phosphate or glucose were preincubated with whole brain homogenates of suckling rats at 37° for 1 hr. Mg2+-ATPase activities were determined according to Bowler & Tirri's (1974). Galactose-1-phosphate or glucose-1-phosphate excessively activated the brain Mg2+-ATPase in a concentration-dependent way. Additionally, galactitol, galactose or glucose stimulated the enzyme up to 35-45% (P4 mM. A mixture of galactose-1-phosphate (2 mM), glactitol (2 mM) and galactose (4 mM), concentrations commonly found in blood and brain of untreated patients with classical galactosaemia, resulted in a 500% enzyme activation (P |
