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
Q

What does Acyl CoA Dehydrogenase do

A

oxidizes the Beta and Alpha carbon to produce FADH2 and trans-enoyl-CoA

26
Q

How many ATP does one mole of Palmitic Acid produce

A

Nets 106 ATP; 2 used in the activation of the Fatty Acid

27
Q

What is the difference of odd numbered fatty acids vs. even number fatty acid Beta Oxidation

A

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
Q

What is the fate of Succinyl-CoA from the Beta Oxidation of Odd Numbered Fatty

A

It enters the TCA Cycle

29
Q

What happens to Unsaturated Fatty Acids in Catabolism

A

Metabolized until unsaturation is reach then a REDUCTASE reduces the double bond and an ISOMERASE moves the disruptive bond

30
Q

Where does breakdown Very Long Chain Fatty Acids

A

Peroxisome

31
Q

What does beta oxidation in the peroxisome produce

A

H2O2; too much is toxic but the peroxisome needs H2O2 to function in immune system

32
Q

What is the key enzyme in the Beta Oxidation of VLCFA

A

Acyl-CoA Oxidase

33
Q

What does Acyl-CoA Oxidase in the peroxisome produce

A

FADH2; which is used to make H2O2. It does not go to the OX-PHOS pathway

34
Q

Metabolic Defects in FA-Oxidation

A

Many enzymes can be defective

35
Q

What happens when the enzymes that cleave Carnitine are defective; in catabolism

A

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
Q

Most common Fatty Acid Enzyme Deficiency in Catabolism

A

Medium Chain Acyl-CoA Dehydrogenase

37
Q

What happens when the Medium Chain Acyl-CoA Dehydrogenase is deficient

A

C8 Fatty Acid accumulates in the liver, poisonous, interferes with urea cycle, elevated levels of ammonia

38
Q

How to treat an MCAD deficiency

A

Patients depend on glucose as an energy source; so dietary treat

39
Q

When are Ketone Bodies Favored

A

When you are in a fasting/starving state

40
Q

What happens when Ketosis is preferred

A

Excessive B-Oxidation of FA’s leading to Increase of Acetyl-CoA; one of the by-products of B-Oxidation

41
Q

What are the three ketone Bodies

A
  1. Acetoacetate
  2. B-Hydroxybutyrate
  3. Acetone
42
Q

What happens when Acetone is built up in the body

A

Acetone breath; sweet

43
Q

What disease, if uncontrolled, leads to a build up of ketones leading to ketoacidosis

A

Diabetes

44
Q

Where are Ketone Bodies produced

A

The mitochondrial matrix of hepatocytes

45
Q

What are Ketone Bodies used for

A

Provide energy in peripheral tissues(muscles), the brain under fasting conditions, and kidneys

46
Q

Energy Yield of 1 mole of Acetoacetate

A

____ ATP - 1 from activation of Acetoacetate

47
Q

Energy Yield of 1 mole B-Hydroxybutyrate

A

_____ ATP - 1 from activation of Acetoacetate

48
Q

Where do Beta-Hydroxybutyrate and Acetone stem from

A

Acetoacetate

49
Q

What happens to Catabolism in the first few hours of fasting

A

gluconeogenesis

50
Q

What happens to Catabolism after 1 day of fasting

A

Triacylglycerols are broken down to release FFA to undergo Beta-Oxidation

51
Q

What happens to Catabolism after 3 days of fasting

A

Ketone bodies made in liver and proteins in the muscles are broken down

52
Q

What happens to Catabolism after 1-2 weeks of starvation

A

brain switches to ketone bodies as major energy source

53
Q

What happens to Catabolism after 2-3 months of starvation

A

Triacylglyercols depleted, protein main source of energy

54
Q

Difference of Physiological ketosis vs. Pathological Ketoacidosis

A

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