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Essential fructosuria is caused by what?
- Absence of fructokinase
- fructose in urine and blood
Hereditary fructose intolerance is caused by what?
- deficiency of aldolase B
- converts f -1- p and f 1,6-BP to dhap and glyceraldehyde
What is the consequence of accumulation of F1-P and F1,6-Bis P in Hereditary fructose intolerance?
- high levels inhibit glycogenlysis and gluconeogenesis
What cells lack sorbitol dehydrogenase?
lens, retina, shwann cells, kidney
What is the consequence of DM in lens, retina, kidney, shwann cells?
accumulated glucose is converted to sorbitol which is osmoticall active and produces peripheral neuropathy, retinopathy and cataract(clouding)
Which organs have sorbitol dehydrogenase?
liver, ovaries and seminal vesicles
When would Hereditary Fructose Intolerance manifest itself?
After breast feeding is stopped
What is the Galactosemia triad?
Cirrhosis, mental retardation, cataract
Classic Galactosemia is caused by what?
Hexokinase can phosphorylate what sugars?
Lactose synthesis occurs where?
What is needed for lactose synthesis
- Glucose converted to UDP-galactose
What forms the lactose synthase complex?
- alpha lactalbumin
- Beta 1,4-Galactosyltransferase
What modulates the activity of beta 1,4 Galactosyltransferase?