Use LEFT and RIGHT arrow keys to navigate between flashcards;
Use UP and DOWN arrow keys to flip the card;
H to show hint;
A reads text to speech;
14 Cards in this Set
- Front
- Back
Galactokinase traps ... as ..., which looks a lot like glucose-1-P
Galactose-1-P is then worked on by ..., which takes it from galactose-1-P to glucose-1-P |
galactose
galactose-1-P galactose-1-P uridylyltransferase |
|
Non-classical galactosemia is a block on ...
Classical galactosemia is a block on the ... |
galactokinase
galactose-1-P uridylyltransferase |
|
when you hear hyperbilirubinemia, think ..., and vice versa
|
jaundice
|
|
Purpose of aldose reductase is to convert glucose to ... (which maintains the osmotic pressure within the lens). If the glucose concentration goes up and stays up for a long period of time, then what will happen is that you will make more ..., and there will be a disruption of the osmotic pressure, and the crystalline proteins will precipitate out --> cataracts.
Most ... will develop cataracts |
sorbitol
sorbitol diabetics |
|
UDP-glucuronate (or UDP glucuronic acid) is necessary to be able to metabolize ...
|
bilirubin
|
|
Bilirubin is the open chain form of ...
|
heme
|
|
UDP glucuronic acid is used for a number of different things:
-bilirubin --> ... (jaundice issue) -steroids, drugs, xenobiotics --> ... (drug metabolism) |
bilirubin diglucuronide
glucuronides |
|
... results from an inability to use the UDP – glucuronic acid
|
Jaundice
|
|
Lactose synthase makes lactose (occurs in females through their ... glands)
Lactose is found in the ... (not blood) A galactosemic female can breast feed her infant and that lactose is not going to be a problem for her because it’s external to her internal organs. |
mammary
gut |
|
when we are talking about the classic form of galactosemia, we are talking about deficiency of what enzyme?
|
galactose-1-P uridyl transferase
|
|
clinitest tablets = tablets that react with ...
|
reducing substance
|
|
Fructose metabolism is the mirror image of ...
Trap fructose using ..., turn it to fructose-1-P, and then there’s an enzyme called ... which makes dihydroxyacetone and glyceraldehyde (glycolytic intermediates that feed into mainstream glucose metabolism) If we have ... deficiency, it’s benign. If we have ... deficiency, it’s a major problem. (very similar to classical galactosemia) |
galactosemia
fructokinase aldolase B fructokinase aldolase B |
|
Cataracts is a key sign and symptom of ..., not ...
|
galactosemia
fructosemia |
|
Hereditary Fructose Intolerance:
Deficiency in fructose-1-phosphate aldolase, aka (...) which Leads to intracellular accumulation of ... causing- Clinical Signs: Infants develop vomiting, hypoglycemia, severe liver and kidney dysfunction. Treatment: Elimination of dietary ... and ... |
aldolase B
fructose-1-phosphate fructose sucrose |