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20 Cards in this Set
- Front
- Back
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two enzymes that can metabolize fructose, what do they form, and where are they found
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hexokinase (most cells) - Fructose-6-P
fructokinase (liver) - F-1-P |
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Aldolase B
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cleaves F-1-P into DHAP and Glyceraldehyde
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essential fructosuria
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fructokinase deficiency
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hereditary fructose intolerance
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Aldolase B deficiency
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why does hyperuricemia and lactic acidosis develop in Aldolase B deficiency
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because F-1-P accumulates and sequesters inorganic phosphate resulting in increased degredation of AMP
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during hyperglycemia in uncontrolled DM, what can accumulate in retina ans Schwann cells
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Sorbitol - because these cells lack significant amounts of sorbitol dehydrogenase (sorbitol --> fructose)
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Galactose-1-P uridyltransferase
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converts G-1-P --> UDP-galactose using UDP-glucose --> glucose-1-P
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what can be used create UDP-galactose in people that are lactose intolerant
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UDP-glucose using UDP-hexose 4-epimerase
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converse galactose --> galactitol
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aldose reductase
*same enzyme converts glucose --> sorbitol |
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Galactokinase deficiency
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galactosemia
galactosuria galactitol accumulation |
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symptoms associated with GALT deficiency
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galactosemia, galactosuria, galactitol accumulation, vomiting, diarrhea, hepatomegaly, jaundice
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inhibits UDP glucose pyrophosphorylase resulting in decreased UDP glucose production
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increased levels of Galactose-1-P
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what does decreased UDP-glucose result in
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kernicterus
impaired brain myelin formation impaired post-translational protein processing growth failure |
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infant presents with galactosemia presents with vomiting and jaundice, these symptoms are related to what metabolite
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Galactose-1-P accumulation
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what is responsible for the cataracts formation in patient with galactosemia
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galactitol
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which metabolite abnormality is responsible for growth delay in patient with 10% G-1-P uridyl transferase activity
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low total pools of UDP-glucose secondary to accumulation of G-1-P
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what health problem is associated with a female patient later in life with classic galactosemia despite appropriate diet restriction
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ovarian failure
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what test can best predict normal intellectual development for an individual diagnosed with galactosemia
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carbon-13 galactose to carbon-13 carbon dioxide breath test
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totally healthy patient presents with positive Clinitest detecting reducing sugars in their urine, what is the most likely defect
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fructokinase
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previously normal and healthy 6-month old breast fed infant begins vomiting 30 minutes after adding fruits to their diet
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Aldolase B deficiency
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