Monosaccharides

Question 1

lactose

• which mono sacs
• dietary source

Answer 1

galatcose and glucose

dairy products

Question 2

Sucrose-which mono sacs

-dietary source

Answer 2

glocuse adn fructose

Table sugar

Question 3

Sorbitol dehydrogenase def

Answer 3

DM-get def in this

• sorbitol is highly hydrophillic-attracts water-cell swells and get cataract
• also in kindey, Schwann cells, lens, retina=places without insulin activity
• retinopahty, cataracts, peripheral neuropathy

Question 4

Hexokinase

Answer 4

Fructose to Fructose 6P

Glucose to Glucose 6P

reverisble

Question 5

Aldolase B and A

Answer 5

Frtuctose 1P to glyceraldehyde and dihydroxyacetone-P

B in liver

A in muscle

Question 6

Aldolase B fructose/glucose metabolism

Answer 6

• rate limiting enzyme of fructose metabolism (not for glycolysis)
• much lower affinity for fructose 1P than fructose 1,6 bisphophsate-very slow at physiolgoc levels of fructose 1P
• @ high dose of fructose-accumulate Fructose 1 P in liver and slowly convert to glycolytic intermediates

Question 7

HFI

• def
• Sx
• TRX

Answer 7

Hereditary fructose intolerance

Aldolase B def

Hypoglycemia, and lactic acidosis, Accumulate higher amounts of fructose 1P in thier livers, Hypoglycemia, vomting, jaundice, hemorrage, hepatomegaly, hyperuicemia

rapid detection adn removal of fructose adn sucrose in diet

Question 8

Essential fructosuria

• Def
• inheritance
• Sx

Answer 8

Lack of fructokinase
AR
Benign=fructosuria

Question 9

Nonclassical galactosemia

• def
• inheritance
• Sx (2)
• Trx

Answer 9

Def in Galactokinase

AR

Elevation of galactose in blood and urine

Causes galactitol accumulation if galactose is presnt in diet-this causes cataracts

Dietary restriction

Question 10

Classical galactosemia
aka
def
inheritance
Sx 
Pathway
Trx

Answer 10

GALT

Def in galactose 1 P uridylyltransferase

AR

Galactosemia, galactosuria, vomiting, diahrea, jaundice

Build up of galactose 1P and galactitol in nerve, lens, liver, and kidney tissues-liver damage, MR, and cataracts

Must remove galatose (and thus lactose from diet-still possiblility to get premature ovarian failure/dev delays

Question 11

Galacosemia Sx

Answer 11

Galactose similar to soribitol-hydrophillic-brings in water to kidney, retina, lens, nerve tissue, seiminal vesicles, ovaries

Question 12

Clinitest

Dipstick

Answer 12

Turns red when have reducing sugars
-these should not be in the urine

If get positive clinitest and negative dipstick for glucose-have another sugar in pee that isnt glucose (aka not diabetes)

Question 13

Reducing sugars def + examples

Answer 13

Sugars with free aldhyde/ketone groups are oxidized to carboxlic acids are known as reducing sugars

glucose, fructose, galatose, maltose, lactose