Metabolism of Disaccharides
Metabolism of Disaccharides
Metabolism of Disaccharides
Site: liver
Galactose metabolism
First the galactose is phosphorylated by
galactokinase to yield galactose-1-p.
Epimerization of galactose-1-phosphate
to G-1-P requires the transfer of UDP
from uridine diphosphoglucose (UDP-
glucose) catalyzed by galactose-1-
phosphate uridyl transferase.
• Effect:
1. Cataract (opacity of eye lens): Galactose is reduced in
the eye by aldose reductase to form galactitol which
accumulates causing cataract
2. Liver failure
3. Mental retardation
4. Galactosuria (excretion of galactose in the urine)