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Galactose Metabolism (Steps (Phosphorylation (catalyzed by (Galactokinase)…
Galactose Metabolism
Steps
Phosphorylation
catalyzed by
Galactokinase
produces
Galactose 1-Phosphate
Uridine diphosphatase-galactose formation
catalyzed by
GALT (galactose 1 phosphate uridyly transferase
produces
Urididine diphosphate Galactose
UDP-galactose conversion to UDP-glucose
catalyzed by
hexose 4 epimerase
produces
UDP-glucose
Disorders
Galactokinase Deficiency
causes
accumulation of galactose in blood and urine
Sxs
galactosemia
galactosuria
cataracts
is caused by
deficiency of galactokinase
Classic Galactosemia
is caused by
deficiency of GALT enzyme
causes
buildup of Galactose 1-Phosphate in tissues
Sxs
galactosemia
galactosuria
accumulation of Galactose 1-phosphate in
liver
brain
eyes
developmental delays
cataracts