FFA
What enzyme breaks down triglycerides to glycerol + free fatty acids?
Lipase
Breakdown of palmitic acid yields (bonus 100 if you can say how much ATP is total)
8 acetyl CoA, 7NADH, 7FADH2, forms 108 total but -2 for activation step
Name the 3 ketone bodies (bonus 100 if you can tell me which one is the first produced)
acetoacetate (first produced), B3 Hydroxybutyrate, Acetone
rate limiting enzyme for B oxidation
CAT 1
When Acyl CoA dehydrogenase is dephosphorylated, is it active or inactive
active!
In the carnitine shuttle, Acyl groups are transferred from CoA to carnitine by what enzyme?
carnitine palmitoyl transferase I (CAT1)
First step of B oxidation (Bonus 100 pts for naming the enzyme) (Bonus 100 pts for the coenzyme)
removal of 2 H atoms via acyl-coA dehydrogenase requiring FAD
What does thiolase do
2 acetyl Coa condensed to form acetoacetyl CoA
What inhibits CAT 1
malonyl CoA
What organ cannot perform B oxidation, requiring ketone bodies when glucose stores are depleted
brain
The conversion of free fatty acids to Acyl-CoA is done by this enzyme
Acyl-CoA synthetase
What does enoyl CoA hydratase do
adds water to saturate the double bond from trans enoyl CoA to form 3 hydroxyacyl CoA
What does HMG CoA synthase do? Why is it important?
add acetyl coA group to form HMG-CoA (6C and intermediate for cholesterol synthesis in the cytosol); IT IS TEH RATE LIMITING ENZYME FOR KETOGENSIS
When I cannot transport Acyl Coa into the mitochondrial membrane, what do I have and the symptoms that follow?
systemic primary carnitine deficiency with hypoketoic and hypoglycemia symptoms
A child is hypoglycemic and was administered 10% dextrose. Blood ammonia was high, liver function tests were slightly elevated, and his serum contained an accumulation of dicarboxylic acids. Only low levels of ketones were detectable in the urine. This infant suffers from?
MCAD deficiency
What does acylcarnitine translocate do? In exchange for what?
carries Acylcarnitine (formed from CAT1 step) to matrix; in exchange for free carnitine
What is B oxidation in peroxisomes?
leads to very long chain FA with acetyl CoA formation and H2O2 broken down by catalase
Starvation-gluconeogenesis is induced depletion of ______
oxaloacetate
A patient presents with myoglobinuria, hypotonia, and weak muscles. Lab results show increased triglycerides in muscles. What deficiency is present?
DKA causes what pathophysiology
Gluconeogenesis induced depletion of oxaloacetate (so does prolonged starvation)
What does CAT 2 do? And what happens as a result?
Acyl group transferred back to CoA to reform acyl-coA. As a result, carnitine released is transported back into IMS via translocate
What happens to odd number fatty acids
oxidized via B oxidation-> propinyl coA-> succinylcholine coA
*propionyl residue from odd chain FA is the only glycogenic part*
How does high alcohol consumption affect gluconeogenesis? What does it change? What symptom will it lead to?
alcoholism changes NADH to NAD ratio (increased NADH, decreased NAD), causes hypoglycemia
A patient is deficient in acyl-CoA dehydrogenase. They have a decreased ability to oxidize fatty acids with 6-10 carbons, which is present in urine. They have hypoglycemia and hypoketonemia, what deficiency is this? What treatment will you advise?
MCAD deficiency (originally reported as sudden infant death or Reye syndrome), treatment includes avoidance of fasting
If oxaloacetate is depleted what cannot happen? Why?
no TCA. first step in TCA combines OAA with acetyl COA