Fatty acid oxidation

Question 1

What type of fatty acid cannot enter the mitochondria?

Answer 1

Long chain fatty acid

Question 2

How do medium and short chain fatty acids enter the mitochondria?

Answer 2

simple diffusion

Question 3

How are fatty acids actiated in the mitochondria?

Answer 3

transformed into Acyl-CoAs

Question 4

Carnitine transport system

Answer 4

Transports acyl-CoAs across the inner mitochondria membrane by turning into acylcarnitine (CPT1) then transferring back to carnitine (CPT2)

Question 5

What does beta-oxidation produce?

Answer 5

1 Acetyl-CoA, 1 FADH2, 1 NADH, an acyl-CoA reduced by 2 carbons

Question 6

Acyl-CoA DH

Answer 6

Makes FADH2 (1st step beta oxidation)

Question 7

Enoyl-CoA hydratase

Answer 7

Adds H2O

Question 8

3HCoADH

Answer 8

makes NADH (3rd step beta oxidation)

Question 9

beta-ketothiolase

Answer 9

Adds HSCoA to make Acetyl Co-A and acyl-CoA

Question 10

How many ATP are formed from FADH2?

Answer 10

2

Question 11

How many ATP are formed from NADH?

Answer 11

3

Question 12

Very long chain acyl-CoA DH prefers how many carbon?

Answer 12

12 to 24

Question 13

Long-chain acyl CoA DH prefers how many carbons?

Answer 13

12 to 16

Question 14

Medium chain Acyl-CoA DH prefers how many carbons?

Answer 14

6 to 8

Question 15

Short chain acyl-CoA DH prefers how many carbons?

Answer 15

4>6>8

Question 16

What inhibits CPT 1?

Answer 16

Malonyl-CoA

Question 17

What happens to beta oxidation when insulin predominates?

Answer 17

Acety-CoA carboxylase is stimulated, Malonyl-CoA concentration increased, so fatty acid synthesis is stimulated while beta-oxidation is inhibited

Question 18

What happens to beta oxidation when glucaogon predominates?

Answer 18

Acetyl-CoA carboxylase is inhibited, Malonyl-CoA concentrations decrease. Inhibition of CPT 1 is relieved.

Question 19

What happens to the product of odd-numbered fatty acid beta oxidation?

Answer 19

propionyl-CoA is converted to succinyl-CoA, which can be used in gluconeogenisis

Question 20

Where does the oxidation of very long chain fatty acids occur?

Answer 20

peroxisomes

Question 21

What is unquie about the modified oxidation in peroxisomes?

Answer 21

oxidation is not linked to cofactor reduction

Question 22

Omega oxidation

Answer 22

Occurs in the ER. Utilizes Cytochrome P450. Is the detox rxn in humands. Produces a dicaroxylic acid

Question 23

What are the 3 ketone bodies?

Answer 23

Acetoacetate, Acetone, beta-hydroxybutyrate

Question 24

HMG-CoA synthase and lysase

Answer 24

Produce only ketone bodies in the liver mitochondria

Question 25

What is the only place ketone synthesis enzymes can be found?

Answer 25

only liver mitochondria

Question 26

What causes the fruity breath in keto-acidosis?

Answer 26

Acetone

Question 27

Medium chain acyl-CoA deficiency

Answer 27

Most common genetic disease of lipid metabolism. Causes non-ketotic hypoglycemia, fatty infiltration of liver, short chain dicarboxylic acids can be found in the urine

Question 28

Carnitine deficiency

Answer 28

Insufficient fatty acid delivery to mitochondria. Causes non-ketotic deficiency. Can treat with oral carnitine

Question 29

CPT I deficiency

Answer 29

LCFA can go into the mitochondria. Hepatomegaly with fatty infiltration. Causes increased plasma carnitine levels. Can be overcome with MCFA

Question 30

Refsum disease

Answer 30

Deficiency in a component of branched-chain oxidation. Serious neurological deficiency. Reduce phytanic acid in diet.