Hepatic Interconversion Of Common Sugars

Front 1

Non-Classical Galactosemia
1) enzyme
2) clinical symtptoms
3) problem

Back 1

1)galactokinase
2)cataracts early in life
3)can't get galactose into cell for utilization, no real problem just pee out reducing sugar

Front 2

Glassical Galactosemia
1) enzyme
2) clinical symtptoms
3) problem

Back 2

1)Galactose-1-P urydiltransferase
2) cataracts, hypoglycemia vomiting & diarrhea folling lactose ingestion,bhyperbilirubinemia, lethargy, liver damage, mental retardation
3) we can get galactose in cell but once there can't convert it to glucose so it's stuck

Front 3

Why hypoglycemia in classical galactosemia?

Back 3

Galactose 1-P is competitive inhibiter to phosphoglucomutase enzyme which converts glucose1P to glucose 6P

Front 4

Why Jaundice in classical galactosemia?

Back 4

UDP-glucuronidate is a tag on biliruben. In classical galactosemia the pathway to make this is obstructed.

Front 5

What is UDP glucuronidate used for?

Back 5

-bilirubin excretion
-drug metabolism

Front 6

Why cateracts in classical & non-classical galctosemia?

Back 6

Aldose reductase converts blood galactose into sorbitol increasing osmotic P in the lens.

Front 7

Females w/ galactosemia can still breast feed infants cuz making not using lactose -T/F?

Back 7

T

Front 8

Where does fructose enter into glycolysis?

Back 8

Glycerol-3-P & DHAP

Front 9

enzymes involved in fructosemia

Back 9

1)fructokinase- benign
2)Aldolase B - problems

Front 10

Aldose B fructosemia

Back 10

lethargy, vomiting,liver damage, hyperbilirubinemia,hypoglycemia, hyperuricemia, renal proximal tubule defect

Front 11

Hereditary fructose intolerance

Back 11

Deficiency in fructose 1-phosphate aldolase
Leads to intracellular accumulation of F1P
Clinically – vomiting, hypoglycemia, severe liver & kidney disfxn
Eliminate dietary fructose & sucrose