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