Agbas: (Reading, Supplemental lecture material, etc.) Flashcards Preview

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Flashcards in Agbas: (Reading, Supplemental lecture material, etc.) Deck (43):
1

Reading Assignment: Disorders of the Krebs Cycle

What are the two disorders discussed in the article?

Fumarase deficiency
2-Oxoglutaric aciduria

2

Reading Assignment: Disorders of the Krebs Cycle


What are the major clinical findings in FUMERASE DEFICIENCY and 2-OXOGLUTARIC ACIDURIA?

-Severe neurologic problems in infants
-Microcephaly
-Mental retardation

3

Reading Assignment: Disorders of the Krebs Cycle

How are fumerase deficiency and 2-oxoglutaric aciduria kreb cycle disorders typically diagnosed?

Urine analysis

-Increased excretion of 2-oxogluterate and fumerase

4

Reading Assignment: Disorders of the Krebs Cycle


How are SUCLA2 and SUCLG1 defects typically diagnosed?

-Dystonia/deafness can indicate
-Direct genetic testing

5

TCA:

Define Amphibolic

Catabolism and anabolism

6

TCA:

How does pyruvate enter the mitochondria?

Mitochondrial Pyruvate Carrier (MPC)

7

TCA:

What enzyme catalyzes the decarboxylation of pyruvate? (*turns pyruvate to acetyl-coa)

Pyruvate dehydrogenase (PDC)

8

TCA:

NADH= ____ ATP

2.5 ATP

9

TCA:

FADH2=___ ATP

1.5 ATP

10

TCA:

What are the products of TCA?

3 NADH
1 FADH2
1 GTP
2 CO2

11

TCA:

What is the enzyme involved in the rate limiting step of TCA?

Isocitrate Dehydrogenase

12

TCA:

What are the 3 enzymes involved in the regulated steps?

1. Citrate Synthase
2. Isocitrate dehydrogenase
3. Alpha-ketogluterate dehydrogenase

13

TCA:

What enzyme produces GTP?

Succinate thiokinase

14

TCA:

Energy status regulates TCA cycle:

When cellular ATP levels are low, the activity of the TCA cycle is ______

Increased

15

TCA:

Energy status regulates TCA cycle:

High levels of cellular ATP triggers the _______ mechanism

Inhibition

16

TCA:

What are anaplerotic reactions?

form intermediates of a metabolic pathway

17

TCA:

What are the two major anaplerotic reactions?

1. Degradation of amino acids
2. Carboxylation of pyruvate

18

TCA:

What amino acids are degraded to create Oxaloacetate?

Asn, Asp

19

TCA:

What amino acids are degraded to create alpha-ketogluterate?

Gln, Pro, His, Arg

20

TCA:

What amino acids are degraded to create Succinyl CoA?

Thr, Met, Ile, Val

These 4 generate Propionyl CoA, which THEN produce Succinyl CoA

21

TCA:

What amino acids are degraded to creat Fumerate?

Phe, Tyr, Asp

22

TCA:

What takes place on the inter-membrane mitochondrion space?

Oxidative phosphorylation

23

TCA:

What takes place in the matrix of the mitochondria?

TCA and fatty acid oxidation

24

TCA:

Redox reactions:
The oxidant is always on the side of the reaction _____

with the electrons

25

TCA:

What is the enzyme that helps eliminate free radicals?

SOD

26

OxPhos:

What are the 3 key goals of OxPhos?

1. Transfer electrons from NADH and FADH2 --> O2
2. To establish a proton gradient across the inner mitochondrial membrane
3. To synthesize ATP

27

OxPhos:

What inhibits ATP synthase/complex 5?

OLIGOMYCIN

28

OxPhos:

If which of the following complexes leave the mitochondria does it indicate apoptosis?

Cyt-c

29

OxPhos:

What happens with the electron transport chain is inhibited?

-Decrease in the pumping protons
-Decrease in the protein gradient
-Inhibition of ATP synthesis

30

OxPhos:

What inhibits COMPLEX I?

Amytal, Rotenone, Myxothiazol, Piericidin A

31

OxPhos:

What inhibits COMPLEX II?

Malonate

32

OxPhos:

What inhibits COMPLEX III?

Antimycin

33

OxPhos:

What inhibits COMPLEX IV?

CO, Cyanide, H2S

34

OxPhos:

What inhibits COMPLEX V/ATP Synthase?

Oligomycin

35

Reduced NADH cannot cross the mitochondrial membrane.

How does it cross?

1. Malate-aspartate shuttle
2. Glycerphosphate shuttle

36

What is the function of:

1. Malate-aspartate shuttle
2. Glycerophosphate shuttle

Transport REDUCED NADH across the mitochondrial membrane

37

Malate-aspartate shuttle:

What organs does it operate in?

Heart, liver and kidneys

38

Malate-aspartate shuttle:

Function?

Transports Reduced NADH into mito-matrix

39

Where does NADH enter the ETC?

Complex I

40

Glycerophosphate-shuttle

What organs does it operate in?

Skeletal muscle, brain

41

Glycerophosphate-shuttle

Function?

Transports FADH2 in the inner mitochondrial membrane

42

Where does FADH2 enter the ETC?

CoQ

43

What was the first diagnosed mitochondrial disease?

Luft's disease