-
Essential fructosuria is caused by what?
- Absence of fructokinase
- benign
- 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
- Hyperuricemia
- Hyperlipidemisa
-
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?
galactose-1-phosphate uridyltransferase
-
Hexokinase can phosphorylate what sugars?
-
Lactose synthesis occurs where?
GA
-
What is needed for lactose synthesis
- Glucose converted to UDP-galactose
- Glucose
-
What forms the lactose synthase complex?
- alpha lactalbumin
- Beta 1,4-Galactosyltransferase
-
What modulates the activity of beta 1,4 Galactosyltransferase?
alpha lactalbumin
|
|
