BCHM - Bioenergetics Flashcards

1
Q

Where does the TCA cycle take place?

A

Inside the mitochondria

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

What is the common pathway into the TCA cycle?

A

Fats, polysaccharides, and proteins are converted into Acetyl-CoA before they can enter the TCA cycle and be used for energy.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

What are the 3 energy equivalent substances generated via the TCA cycle?

A

NADH, FADH2, and GTP

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

Describe how Acetyl-CoA is derived from carbohydrates.

A

Glucose (6 carbons), gets oxidized during glycolysis into 2 pyruvates (3 carbons). The 2 pyruvates are then decarboxylated into 2 Acetyl Co-A molecules for TCA cycle use

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

Describe how Acetyl CoA is derived from lipids.

A

Triacylglycerols are converted into fatty acids. The fatty acids then undergo beta-oxidation to form Acetyl CoA

The oxidation of ketone bodies also forms Acetyl CoA

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

Describe how Acetyl CoA is derived from proteins.

A

Proteins are broken down into amino acids. The amino acids are then turned into Acetyl CoA

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

What does PDC stand for?

A

Pyruvate dehydrogenase complex

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

When PDC is phosphorylated, is it in its active or inactive form?

A
Phospho = INACTIVE
Dephospho = ACTIVE
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

Where in the PDC does phosphorylation occur?

A

Phosphorylation occurs in coenzyme Thiamine pyrophosphate (TPP) of the E1 complex

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

The PDC is made up of E1, E2, and E3 subunits. What are each of the subunits comprised of?

A

E1 = TPP
E2 - Lipoic acid and CoA
E3 = NAD+ and FAD

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

What enzyme ACTIVATES the PDC?

A

Pyruvate dehydrogenase phosphatase (PDP)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

What are the activators of pyruvate dehydrogenase phosphotase, and thus activate the PDC?

A

Ca2+ and Mg2+ activate PDP

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

What acts as inhibitory signals of the active state E1 subunit of the PDC?

A

Acetyl CoA and NADH

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

What molecules inhibit the deactivation of the PDC?

A

Pyruvate, CoA, NAD+, and ADP

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

What molecules cause the deactivation of pyruvate dehydrogenase kinase?

A

Acetyl CoA, NADH, and ATP

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

What enzyme changes PDC from being active to inactive?

A

Pyruvate dehydrogenase kinase (PDK)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
17
Q

In a phosphate deficiency, what happens to the PDC?

A

It remains phosphorylated, aka inactive. This leads to glucose being converted to lactate instead of Acetyl CoA

Buildup of lactate causes lactic acidosis, resulting in CNS effects

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
18
Q

With a phosphate deficiency, what amino acid should be avoided?

A

Alanine should be avoided and a ketogenic diet is strongly recommended

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
19
Q

In what steps of the TCA cycle is NADH produced?

A

Isocitrate to alpha-ketoglutarate (isocitrate dehydrogenase)

Alpha-ketoglutarate to Succinyl-CoA (alpha-ketoglutarate dehydrogenase)

Malate to oxaloacetate (malate dehydrogenase)

TOTAL OF THREE NADH PRODUCED EACH CYCLE

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
20
Q

In what step of the TCA cycle is GTP produced?

A

Succinyl-CoA to Succinate (Succinyl-CoA dehydrogenase)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
21
Q

In what step of the TCA cycle is FADH2 produced?

A

Succinate to fumarate (succinate dehydrogenase)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
22
Q

How many ATP are produced by each NADH, FADH2, and GTP?

A
NADH = 2.5 ATP
FADH2 = 1.5 ATP
GTP = 1 ATP
23
Q

What is the rate limiting step of the TCA cycle?

A

Isocitrate to alpha-ketoglutarate via the enzyme isocitrate dehydrogenase

24
Q

What is the function of the pyruvate dehydrogenase complex?

A

Convert Pyruvate into Acetyl-CoA for entrance into the TCA cycle

25
Q

Acetyl CoA combines with what molecule to form citrate?

A

Oxaloacetate generated via the TCA cycle

26
Q

When cellular levels of ATP are low, what happens to the TCA cycle?

A

Increased activity of TCA cycle to produce more ATP

26
Q

When cellular levels of ATP are low, what happens to the TCA cycle?

A

Increased activity of TCA cycle to produce more ATP

27
Q

What are the two major anaplerotic reactions in the TCA cycle?

A

Degradation of amino acids and carboxylation of pyruvate

28
Q

What amino acids are degraded to replenish alpha-ketoglutarate?

(5 of them)

A

Glutamine, proline, histidine, arginine, and glutamate

29
Q

What amino acids degrade to form Succinyl CoA in the TCA cycle?

(4 of them)

A

Threonine, methionine, isoleucine, and valine

The metabolic byproducts for propionyl CoA, which then replenishes Succinyl CoA

30
Q

What amino acids are degraded to form fumarate in the TCA cycle?

(3 of them)

A

Phenylalanine, tyrosine, and aspartate

31
Q

What amino acids are degraded to form oxaloacetate in the TCA cycle?

(2 of them)

A

Asparagine and aspartate

32
Q

Describe 2-Oxoglutaric aciduria (Alpha-ketoglutaric acid)

A

Rare disorder displaying developmental delay and severe neurologic problems in infants including metabolic acidosis, microcephaly, and intellectual disability

33
Q

Describe Fumarase deficiency

A

Severe neurological impairment. Fatal outcome within first 2 years of life
Encephalomyopathy, dystonia, urinary excretion of TCA intermediates are common

34
Q

What three key goals must be accomplished for a successful OxPhos?

A
  1. transfer of electrons from NADH and FADH2 to O2
  2. establish proton gradient across INNER membrane and intermembrane space
  3. Synthesize ATP
35
Q

In the TCA cycle, the first goal is the transfer of electrons. Describe how this happens

A

Electrons flow from molecules with low Standard Redox Potential to those with the highest.

36
Q

What causes the pumping of H+ through the mitochondrial inner membrane?

A

Electron transfer through the respiratory chain.

37
Q

What two factors constitute a proton-motive force (pmf) to drive ATP synthesis by Complex V?

A
  1. pH gradient

2. membrane potential

38
Q

How many H+ molecules are required to form 1 ATP via the ATP Synthase (Complex V)?

A

4 H+ required to form 1 ATP via Complex V

39
Q

What molecule inhibits proton transport through Complex V?

A

Oligomycin

40
Q

What inhibits Complex I?

A

Amytal, Rotenone, Myxothiazol, and Piericidin A

ARM Pit

41
Q

What inhibits Complex II?

A

Malonate

42
Q

What inhibits Complex III?

A

Antimycin

43
Q

What inhibits Complex IV?

A

CO, Cyanide, and H2S

44
Q

What inhibits Complex V?

A

Oligomycin

45
Q

What inhibits Complex V?

A

Oligomycin

46
Q

A high ATP/ADP ratio will do what?

A

Decrease ATP concentrations

47
Q

A low ATP/ADP ratio will do what?

A

Increase ATP concentration

48
Q

What occurs during uncoupling in OxPhos?

A

Protons uncouple from their carrier electrons and reenter the mitochondrial matrix. The TCA cycle is accelerated and ATP synthase is inhibited (no ATP synthesis). There is heat generation

49
Q

What are the two mechanisms of action for Superoxide Dismutase?

A
  1. Cu/Zn SOD (SOD1) - cytosolic

2. Mn/Zn SOD (SOD2) - mitochondrial

50
Q

In what organs is the Malate-Aspartate Shuttle active?

A

Heart, liver, and kidneys

51
Q

What is the function of the malate-aspartate shuttle?

A

Generates NADH into the mitochondrial matrix and pumps NADH through the electron transport chain at Complex 1

52
Q

What organs is the glycerophosphate-shuttle active?

A

Skeletal muscle and the brain

53
Q

What is the function of the glycerophosphate-shuttle?

A

Generate FADH2 in the inner mitochondrial membrane and join into the ETC at CoQ