Beta Oxidation & Ketogenesis

Question 0

How is HS lipase regulated?

Answer 0

Inhibited by insulin. Activated by ABSENCE of insulin. Activated by epinephrine (phosphorylation)

Question 1

What enzyme cleaves TAGs to remove the FA from storage?

Answer 1

Hormone sensitive lipase

Question 2

How are free fatty acids transported in serum?

Answer 2

Albumin

Question 3

Where do free fatty acids in blood go?

Answer 3

Liver skeletal and cardiac muscle

Question 4

What is the rate limiting step of beta oxidation?

Answer 4

HS lipase!

Question 5

Where is the glycerol sent after being released from TAGs?

Answer 5

Liver! For Gluconeogenesis or glycolysis

Question 6

Does the brain use fatty acids for metabolism?

Answer 6

Nope!

Question 7

What fatty acids pass the brain blood barrier?

Answer 7

Omega 3 and omega 6! Used in membranes (arachadonic acid a DHA)

Question 8

What findings are associated with MCAD deficiency?

Answer 8

Decreased BOx ; reliance on glucose; HYPOGLYCEMIA. Decreased formation of acetyl CoA; HYPOKETONEMIA

Question 9

What organ is CPT1 deficiency associated with?

Answer 9

Liver! High serum carnitine levels

Question 10

What organ is associated with CPT2 deficiency?

Answer 10

Skeletal muscle and heart muscle. Myoglobinuria and elevated ckmm following prolonged exercise

Question 11

What are the symptoms of primary carnitine deficiency?

Answer 11

Hypoglycemia (impaired Gluconeogenesis) potentially weakness and cardiomyopathy

Question 12

What is the cause of Jamaican vomiting sickness?

Answer 12

Consumption of unripe ackee fruit. Contains hypoglycin A an inhibitor of MCAD. Hypoglycemia coma death