Metabolism of mono and disaccharides

  1. Essential fructosuria is caused by what?
    • Absence of fructokinase
    • benign
    • fructose in urine and blood
  2. Hereditary fructose intolerance is caused by what?
    • deficiency of aldolase B
    • converts f -1- p and f 1,6-BP to dhap and glyceraldehyde
  3. 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
  4. What cells lack sorbitol dehydrogenase?
    lens, retina, shwann cells, kidney
  5. 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)
  6. Which organs have sorbitol dehydrogenase?
    liver, ovaries and seminal vesicles
  7. When would Hereditary Fructose Intolerance manifest itself?
    After breast feeding is stopped
  8. What is the Galactosemia triad?
    Cirrhosis, mental retardation, cataract
  9. Classic Galactosemia is caused by what?
    galactose-1-phosphate uridyltransferase
  10. Hexokinase can phosphorylate what sugars?
    • Glucose
    • Mannose
    • Fructose
  11. Lactose synthesis occurs where?
  12. What is needed for lactose synthesis
    • Glucose converted to UDP-galactose
    • Glucose
  13. What forms the lactose synthase complex?
    • alpha lactalbumin
    • Beta 1,4-Galactosyltransferase
  14. What modulates the activity of beta 1,4 Galactosyltransferase?
    alpha lactalbumin
Card Set
Metabolism of mono and disaccharides
Metabolism of mono and disaccharides