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MCM- Lipid Catabolism Flashcards

1
Q

How many phases of fatty acid breakdown are there

A

2 phases

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2
Q

What is Phase 1 of Fatty Acid Breakdown

A

Transport of free fatty acids into the mitochondrial matrix

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3
Q

What is Phase 2 of Fatty Acid Breakdown

A

Beta-Oxidation

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4
Q

What shuttle transports free fatty acids into the mitochondrial matrix

A

Carnitine Shuttle

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5
Q

Two Sources of Fatty Acids

A

Dietary Fats and Stored Fats

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6
Q

Where does Fatty Acid Activation Occur

A

The Cytosol

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7
Q

What is bound to Fatty Acids in the cytosol

A

Albumin

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8
Q

What is Albumin’s goal when bound to Fatty Acids

A

Keep it soluble

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9
Q

Once activated what is the form of the Fatty Acid

A

Fatty Acyl-CoA

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10
Q

Can Fatty acyl-CoA get into the Mitochondria

A

No; the outside membrane of the mitochondria is impermeable to Fatty Acyl-CoA

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11
Q

Why does Fatty Acid Activation need to occur

A

Because Fatty Acids bound with albumin are impermeable to the plasma membrane

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12
Q

What happens to Fatty Acyl-CoA to get into the inner membrane

A

Carnitine is added and CoA is detracted making Fatty Acyl-Carnitine

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13
Q

What happens to Fatty Acyl Carnitine once it is in the mitochondrial matrix of the mitochondria

A

Carnitine is removed and Fatty Acyl-CoA is remade

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14
Q

What happens to Fatty Acyl-CoA once it is remade in the Mitochondrial Matrix

A

Beta-Oxidation

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15
Q

Which Fatty Acids can diffuse into the Mitochondria

A

Short and Medium Chain

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16
Q

What is the rate limiting step of the Carnitine Shuttle

A

Carnitine Palmitoyltransferase-1; CPT1

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17
Q

What inhibits Carnitine Palmitoyltransferase-1

A

Malonyl-CoA

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18
Q

Where is the Carnitine Palmitoyltransferase-1 located

A

In the mitochondrial intermembrane space

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19
Q

What are the products of Beta Oxidation and where do they go

A

Acetyl-CoA; enters the TCA cycle
FADH2; delivers electrons to Ubiquinone of ETC
NADH; delivers electrons to Complex 1 of ETC

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20
Q

How many steps of Beta Oxidation are there

A

Four

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21
Q

What are the four steps of Beta Oxidation

A
  1. Oxidation
  2. Hydration
  3. Oxidation
  4. Thiolysis
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22
Q

What enzyme performs step 1 of Beta Oxidation

A

Acyl CoA Deydrogenase; Oxidate

23
Q

What is the rate limiting step of Beta Oxidation

A

Acyl-CoA Deydrogenase (ACAD)

24
Q

What are the four reactions of Beta Oxidation in order

A
  1. Oxidation
  2. Hydration
  3. Oxidation
  4. Thiolysis
25
What does Acyl CoA Dehydrogenase do
oxidizes the Beta and Alpha carbon to produce FADH2 and trans-enoyl-CoA
26
How many ATP does one mole of Palmitic Acid produce
Nets 106 ATP; 2 used in the activation of the Fatty Acid
27
What is the difference of odd numbered fatty acids vs. even number fatty acid Beta Oxidation
Odd numbered fatty acids are metabolized until a 3 C fatty acid (Propionyl-CoA). Propionyl-CoA is then converted through two different enzymes to Succinyl-CoA
28
What is the fate of Succinyl-CoA from the Beta Oxidation of Odd Numbered Fatty
It enters the TCA Cycle
29
What happens to Unsaturated Fatty Acids in Catabolism
Metabolized until unsaturation is reach then a REDUCTASE reduces the double bond and an ISOMERASE moves the disruptive bond
30
Where does breakdown Very Long Chain Fatty Acids
Peroxisome
31
What does beta oxidation in the peroxisome produce
H2O2; too much is toxic but the peroxisome needs H2O2 to function in immune system
32
What is the key enzyme in the Beta Oxidation of VLCFA
Acyl-CoA Oxidase
33
What does Acyl-CoA Oxidase in the peroxisome produce
FADH2; which is used to make H2O2. It does not go to the OX-PHOS pathway
34
Metabolic Defects in FA-Oxidation
Many enzymes can be defective
35
What happens when the enzymes that cleave Carnitine are defective; in catabolism
Carnitine will build up in the transmembrane space; leading to a carnitine deficiency since Fatty Acyl-Carntine cant diffuse into the matrix through the inner membrane and it can go back out but stops after the outter membrane
36
Most common Fatty Acid Enzyme Deficiency in Catabolism
Medium Chain Acyl-CoA Dehydrogenase
37
What happens when the Medium Chain Acyl-CoA Dehydrogenase is deficient
C8 Fatty Acid accumulates in the liver, poisonous, interferes with urea cycle, elevated levels of ammonia
38
How to treat an MCAD deficiency
Patients depend on glucose as an energy source; so dietary treat
39
When are Ketone Bodies Favored
When you are in a fasting/starving state
40
What happens when Ketosis is preferred
Excessive B-Oxidation of FA's leading to Increase of Acetyl-CoA; one of the by-products of B-Oxidation
41
What are the three ketone Bodies
1. Acetoacetate 2. B-Hydroxybutyrate 3. Acetone
42
What happens when Acetone is built up in the body
Acetone breath; sweet
43
What disease, if uncontrolled, leads to a build up of ketones leading to ketoacidosis
Diabetes
44
Where are Ketone Bodies produced
The mitochondrial matrix of hepatocytes
45
What are Ketone Bodies used for
Provide energy in peripheral tissues(muscles), the brain under fasting conditions, and kidneys
46
Energy Yield of 1 mole of Acetoacetate
____ ATP - 1 from activation of Acetoacetate
47
Energy Yield of 1 mole B-Hydroxybutyrate
_____ ATP - 1 from activation of Acetoacetate
48
Where do Beta-Hydroxybutyrate and Acetone stem from
Acetoacetate
49
What happens to Catabolism in the first few hours of fasting
gluconeogenesis
50
What happens to Catabolism after 1 day of fasting
Triacylglycerols are broken down to release FFA to undergo Beta-Oxidation
51
What happens to Catabolism after 3 days of fasting
Ketone bodies made in liver and proteins in the muscles are broken down
52
What happens to Catabolism after 1-2 weeks of starvation
brain switches to ketone bodies as major energy source
53
What happens to Catabolism after 2-3 months of starvation
Triacylglyercols depleted, protein main source of energy
54
Difference of Physiological ketosis vs. Pathological Ketoacidosis
Ketosis; mild to moderate increase in ketone bodies Ketoacidosis; massive accumulation of ketone bodies, occurs when glucagon/insulin ratio is increased, favoring fatty acid breakdown. Glucagon too high because insulin isnt receptive. Breaksdown all TAG supply and switches to ketone bodies

Biochemistry (7 decks)

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