Beta Oxidation Of Fatty Acids

Question 0

In plasma, longer-chain FFA arecombined with 1 _____, and in the cell they are at-tached to a 2._____, so that in factthey are never really “free.”

Answer 0

1. Albumin

2. fatty acid-binding protein

Question 1

Rate limiting step in fatty acid oxidation

Answer 1

Carnitine palmitoyl transferase

Question 2

Long-chain acyl-CoA (or FFA) will not penetratethe inner membrane of mitochondria. However, _______, present in the outermitochondrial membrane, converts long-chain acyl-CoA to acylcarnitine, which is able to penetrate theinner membrane and gain access to the β-oxidationsystem of enzymes

Answer 2

car-nitine palmitoyltransferase-I via carnitine shuttle

Question 3

Fatty acids with an odd number of carbon atoms are oxi-dized by the pathway of β-oxidation, producing acetyl-CoA, until a three-carbon (_______) residue re-mains.

Answer 3

propionyl-CoA

Question 4

________ is caused by eating theunripe fruit of the akee tree, which contains a toxin,hypoglycin, that inactivates medium- and short-chainacyl-CoA dehydrogenase, inhibiting β-oxidation andcausing hypoglycemia

Answer 4

Jamaican vomiting sickness

Question 5

________ is a rare neurologic disor-der due to a defect that causes the accumulation of phy-tanic acid, which is found in plant foodstuffs andblocks β-oxidation.

Answer 5

Refsum’s disease