Powerpoints Flashcards
What is hereditary fructose intolerance caused by?
aldolase B
What intermediate in glycolysis will be passed in the presence of arsenate?
13bPG
Where does hexokinase convert fructose to F6P?
muscle
Where does fructokinase convert fructose into F1P?
liver
What is the function of aldolase B?
convert F1P into glyceraldehyde
What is the definition of galactosemia?
failure to use galactose in glycolysis
Newborns presenting with galactosemia present with what?
milk intolerance and signs of liver failure
What is the main regulated step of glycolysis?
PFK I
Where is the glycerol phosphate shuttle located?
brain and muscle
Where is the malate-aspartate shuttle located?
liver and heart
Can the body synthesize glucose from fat?
No
What does acetyl-CoA do in relation to carbohydrate metabolism?
inhibits PDH
activates pyruvate carboxylase
What are the products of the non-oxidative phase of the pentose phosphate pathway?
F6P and GA3P
What drug are patients with G6P dehydrogenase inactivity sensitive to? and what food?
primaquine and fava beans
Thiamine affects what type of reactions of the PPP?
transketolase reactions
What activated sugar does UDP-glucose pyrophosphorylase act on?
G1P
Where are proteoglycans degraded?
lysosomes
What are the most abundant saccharides attached to proteins?
mannose, glucose and fucose
What are two hallmarks of defective FA uptake?
hypoglycemia and low ketone body synthesis
How many ATP can an acetyl-CoA molecule yield?
10 each
Palmitic acid yields?
10 acetyl-CoA
7 NADH and FADH2
What are two hallmarks of peroxisomal beta-oxidation?
preferentially degrade long chain FAs and produce H2O2
What enzyme deficiency sometimes causes acute fatty liver of pregnancy?
LCHAD
What else can cause AFLP?
fetus releasing hydroxyacyl metabolites
