Other sugars Flashcards
What accumulates in classic Galactosemia? S/s?
Galactitol
Vomiting, diarrhea, hypoglycemia
Cataracts (within few days of birth), hepatosplenomegaly, mental retardation
Lactose monosaccharide components?
Galactose + glucose = lactose
Enzyme missing in classic Galactosemia
Galactose 1-P uridyltransferase
Sucrose monosaccharide components
Fructose + glucose = sucrose
Fastest metabolism among the sugars
Fructose
Enzyme missing in fructose intolerance
Aldolase B
Enzyme missing in essential fructosuria
Fructokinase
Aldolase A vs B
A: for glycolysis
B: for fructose metabolism
S/s of essential fructosuria
Asymptomatic
Fructose in blood and urine
Enzyme that converts glucose to sorbitol
Aldose reductase
Sorbitol attracts water by osmosis (cataracts, Peripheral neuropathy)