new_n_lost
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Galactokinase Def Cant produce Galactose-1-Phosphate which causes the Most damage in Galactose-1-Phosphate Uridyltransferase (GALT) deficiency.
GK def still causes Cataracts bcos of Aldose Reductase Enzyme Presence. When presented with accumulated galactose, this enzyme reduces the aldehydic end group and produces galactitol, the analogous sugar alcohol. This compound exerts osmotic pressure within the lens because it diffuses very slowly.
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| edie
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 yep, it's that galactose-1-P that wreaks havoc on liver, among other things.
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| usmletopper1
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in galactokinase defeciency-unphosphorylated galotose accumulates
in GIPUT defeciency-phosphorylated galactose accumulates,once the sugar is phosphorylated it kind of gets trapped inside the cell annnd cannot come out,thus serious and more manifestations compared to galactokinase defeciency.
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| sfk
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ok so the serious manifestations are because of accumulation of the gal 1P, not galctose or galactitiol.
by analogy, in frucose intolerance, fructokinase deficiency produces only a benign fructosuria and Aldolase def causes the more severe manifestations due to fructose 1-P accumulation. am i right?
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| new_n_lost
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yup something like u can say..... but its entirely a different mechanism.
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| GOGETA
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I saw a question about that in pediatrics
nnl if you score nore than 250 you will become FORUM SUPER HERO, now you are SUPER HERO in my book
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| new_n_lost
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Dude Thanks for the Vote of Confidence I sure hope so to get such a score.
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FORUM RULES-- Those who believe in telekinesis, raise my hand. I get enough exercise just by pushing my luck --P4U World.." The pure and simple truth is rarely pure and never simple."
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| sfk
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entirely different mecahnism? could u throw some more light?
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| new_n_lost
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In Fructose Metabolism the Def. of Aldolase B leads to Accumulation of Fructose along with Fructose-1-Phosphate. Now Galactose in itself doesnt cause any harm but the Fructose Alone leads to the Trapping of Phosphorus inside the Cells and thus causing Hypophosphatemia which leads to dec in ATP and Inc AMP which causes accelerated Uric Acid Production. This is Alone caused by Fructose yet there is Fructose 1Phosphate which leads to Severe Liver Damage along with Dec Lactic Acid Excretion in Renal Tubules.
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FORUM RULES-- Those who believe in telekinesis, raise my hand. I get enough exercise just by pushing my luck --P4U World.." The pure and simple truth is rarely pure and never simple."
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| new_n_lost
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I Hope i have made it simpler for u to understand otherwise do let me know i will give it another shot. 
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| sfk
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"Galactose in itself doesnt cause any harm but the Fructose Alone leads to the Trapping of Phosphorus inside the Cells "
i believe its both fructose 1-P and gal 1-P that cause Pi depletion and consequent hyperuricemia, which accumlate in aldolase n GPUT deficiency respectively.
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| new_n_lost
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Ur Reference Plz Cos i havent Chked Lippincott yet but i had searched this when i was doing my own Study From NEJM.
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FORUM RULES-- Those who believe in telekinesis, raise my hand. I get enough exercise just by pushing my luck --P4U World.." The pure and simple truth is rarely pure and never simple."
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| sfk
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read about fructose 1P in Lippincott, n gal 1P in kaplan q book. havent read NEJM!!!
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