Fatty Acid Metabolism

Question 1

Citrate is a powerful activator of _____-___ synthesis.

Answer 1

Fatty Acid

Question 2

During the Citrate shuttle cycle, it is the conversion of Malate to pyruvate via Malic enzyme that generates ______, which is essential to fatty synthesis.

Answer 2

NADPH

Question 3

Seeing _____ acids build up than you know there’s a MCAD or SCAD deficiency

Answer 3

Dicarboxylic

Question 4

To get Acetyl CoA from the mitochondrion into the Cytoplasm you to first turn into ____ to take the shuttle.

Answer 4

Citrate

Question 5

The two regulators of Acetyl-CoaA-Carboxylase are ______, which makes sense considering the shuttle that’s used, and ______, whiich is the product.

Answer 5

• Citrate

- Long Chain Fatty Acyl-CoA

Question 6

Odd-chain fatty acids are the one exception where glucose can be made because Propionyl-CoA become ______ with Methylmalonyl-CoA which goes into the TCA Cycle and can glucneo.

Answer 6

Succinyl-CoA

Question 7

In MELAS you would expect _____ Lactate and NADH than in PDH

Answer 7

More

Question 8

Beta Oxidation removes ____ carbons at a time

Answer 8

2

Question 9

Less Oxaloacetate slows down the TCA, and so more of it gets shunted into gluconeo, creates more Acetyl-CoA that get shunted and create ____ ____

Answer 9

Ketone Bodies

Question 10

Hypoketotic hypoglycemia, Hyperammonemia, and a build up of Dicarboxylic acids means there’s an impairment of _________ oxidation

Answer 10

Medium chain fatty acid oxidation

Question 11

Short and Medium chain fatty acids can diffuse into the mitochondria, but Long-chain fatty acids use the _____ cycle for mitochondrial transport.

Answer 11

Carnitine

Question 12

After you have acyl co-a (4C), you load another ______ CoA (3C) onto FAS and lose another CO2 from it, resulting in the +2Carbons for each addition.

Answer 12

Malonyl

Question 13

The rate limiting step for Fatty acid synth is ________ Carboxylase

Answer 13

Acetyl-CoA

Question 14

FFA is converted to Acyl-CoA, which inhibits _____, (1st enzyme for FA formation) resulting in low malonyl CoA concentration in the liver.

Answer 14

Acetyl CoA Carboxylase

Question 15

How much energy does Acetone provide?

Answer 15

None

Question 16

The _____ Shuttle allows the synthesis of NADPH and the recycling of Oxaloacetate between cytoplasm and mitochondria

Answer 16

Malate

Question 17

Insulin helps _______ turn into Fatty acids, so in diabetics the buildup of this molecule results in Ketone Bodies.

Answer 17

Acetyl-CoA

Question 18

Refsum’s disease is a defect with ______ which causes neurological problems

Answer 18

Alpha-oxidation

Question 19

In the first step of Fatty Acid Synthase ______ and ______ are attached onto FAS

Answer 19

• Acetyl Co-A (2C)

- Malonyl Co-A (3C)

Question 20

Ketone bodies are mainly made in the Liver and in the organelle ______, whereas cholesterol is made in the Cytosol and ER

Answer 20

Mitochondria

Question 21

With a Carnitine Shuttle Defect, you would expect the Fatty Acid levels in the blood to _____ and you’ll see Hypoketotic Hypoglycemia specifically with CPT-I

Answer 21

Rise

Question 22

Acetyl CoA Carboxylase follows the same insulin/glucaon pattern, so the hormone _____ activates it by dephosphorylating via Protein phosphatase.

Answer 22

Insulin

Question 23

Fatty Acyl-coA becomes Acylcarnitine with CPT-I. Considering that _______ is needed to start Fatty synthesis; ______ inhibits CPT-I because you want to create and not store.

Answer 23

Malonyl CoA

Question 24

Add a carbon to odd-chain fatty acid with _______ that uses Biotin

Answer 24

ABC Carboxylase

Question 25

Glucagon and Epinephrine uses PKA to phosphorylate the protective protein _______ which allows access of lipase to TGs and also phosphorylates HSL to mobilize fatty acids.

Answer 25

Perilipin

Question 26

Acyl-CoA dehydrogenase defiency causes Hypoglycemia in times of fasting because of lack of ______.

Answer 26

Gluconeogenesis

Question 27

The liver cannot use Acetoacetate as energy because it lacks ________

Answer 27

Thiophorase, or CoA transferase

Question 28

_____ fats generate more energy than _____ fats because the first step via Acyl Co-A dehydrogenase makes that initial FADH2

Answer 28

Saturated, Unsaturated

Question 29

Propionyl-CoA is made in amino acid recycling and odd-chain fatty acid degradation. A defect in _______ Co-A Mutase causes a buildup and does not allow proper utilization.

Answer 29

Methylmalonyl

Question 30

The two transcription factors that promote ACC expression are _____, activated by carbs, and ______, activated by insulin

Answer 30

- Carbohydrate Response Element Binding Protein (ChREBP) | - SREBP-1c

Question 31

4 main biochemical steps for FA synthase, as it grows 2 carbons at a time with 7 acetyl coAs.

Answer 31

1. Condensation 2. Reduction 3. Dehydration 4. Reduction (Love Hydrogens, Hate Water)