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MCM - Molecular and Cellular Mechanisms) > TCA cycle and the Electron Transport Chain > Flashcards

TCA cycle and the Electron Transport Chain Flashcards

1
Q

Regulatory enzymes in PDH complex

A

PDH kinase and PDH phosphatase

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2
Q

The 5 Co-enzymes in the PDH complex

A

E1- TPP
E2- lipoic acid and CoA
E3- FAD and NAD+

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3
Q

PDH complex is regulated by _______ modifications

A

Covalent

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4
Q

PDH kinase and PDH phosphatase regulate PDH complex as follows:

A

Kinase phosphorylates and deactivates

Phosphatase dephosphorylates and activates

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5
Q

PDH Kinase is allosterically activated by:

A

ATP, Acetyl CoA and NADH

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6
Q

PDH Kinase is allosterically inhibited by:

A

Pyruvate

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7
Q

PDH phosphatase activiator:

A

Ca2+

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8
Q

Citrate synthase is inhibited by:

A

Citrate

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9
Q

Acontinase is inhibited by:

A

Flouroacetate (rat poisoning)

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10
Q

Isocitrate dehydrogenase is inhibited by

A

ATP, NADH

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11
Q

Isocitrate dehydrogenase is activated by

A

ADP

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12
Q

Rate limiting enzyme of TCA cycle

A

Isocitrate dehydrogenase

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13
Q

Coenzymes required for alpha-ketoglutarate dehydrogenase complex

A

TPP, lipoic acid, FAD, NAD+ and CoA

Mnemonic: TLC For Nancy

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14
Q

Alpha-ketoglutarate dehydrogenase complex is inactivated by

A

NADH, Succinyl CoA

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15
Q

Alpha-ketoglutarate dehydrogenase complex is activated by

A

Ca2+

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16
Q

Arsenite inhibits _____ and _____

A

lipoic acid and alpha-KG

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17
Q

Arsenic poisoning clinical findings:

A

Rice watery stools, vommiting, prolonged QT, garlicy smelling breath

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18
Q

B1 (thiamine) deficiency >

A

TPP deficiency > Wernicke-Korsakoff syndrome in alcoholics > wernicke encephalopathy > ataxia, confusion and opthalmopelgia

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19
Q

B2 (riboflavin) deficiency >

A

FAD > Cheilosis > Inflammation of lips, scaling at corners of mouth, dermatitis, corneal vascularization

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20
Q

B3 (niacin) deficiency >

A

NAD+ > Pellagra > Diarrhea, dermatitis, dementia

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21
Q

B6 (benzoic acid) >

A

Coenzyme A > Fatigue, sleep disturbances, impaired coordination > alopecia > dermatitis > enteritis > adrenal insufficiency

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22
Q

Overall regulation of the TCA cycle:

Inhibitors and activators

A

Inhibitors - ATP, NADH, Succinyl CoA, Citrate

Activators: ADP, AMP, Ca2+

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23
Q

Energy yield of TCA cycle:

A

12 ATP per Acetyl CoA or 24 per glucose mol

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24
Q

Citrate is a precursor for:

A

Fatty acid synthesis

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25
Q

alpha-keyoglutarate is a precursor for:

A

AA synthesis

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26
Q

Succinyl CoA is a precursor for:

A

Heme synthesis

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27
Q

Malate is a precursor for:

A

Gluconeogenesis

28
Q

Oxaloacetate is a precursor for:

A

AA synthesis

29
Q

Anaplerotic reaction

A

Pathways that replenish the intermediates of the TCA cycle

30
Q

5 important anaplerotic reactions

A

Pyruvate carboxylase - produces Oxaloacetate from pyruvate

Glutamate > alpha-ketoglutarate

Leucine/isoleucine > Succinyl CoA

Tyr/phe > Fumarate

Aspartate > oxaloacetate

31
Q

Pyruvate carboxylase deficiency

A

Causes lactic acidosis, seizures, muscle weakness, ataxia

32
Q

Pyruvate carboxylase is activated by

A

Acetyl CoA

33
Q

Pyruvate carboxylase requires _____ as a cofactor

A

Biotin

34
Q

Formation of ATP through the build up of H+ gradient in mitochondrial intermembrane space

A

Chemiosmotic coupling

35
Q 
Complex I
Complex II
\_\_\_\_\_\_\_\_
Complex III
\_\_\_\_\_\_\_\_
Complex IV
Complex V
A 
I - NADH dehydrogenase 
II - Succinate dehydrogenase
Co enzyme Q
III - Cytochrome C reductase 
Cytochrome C
IV - Cytochrome C oxidase
V - ATP synthase
36
Q

NADH/NAD+ and FAD/FADH2 are prosthetic groups for which complexes in the ETC?

A

I and II

37
Q

Heme groups are prosthetic groups for which complexes in the ETC?

A

III and IV

38
Q

Iron sulfur complexes are prosthetic groups for which complexes in the ETC?

A

I, II, III

39
Q

Copper ions are prosthetic groups for which complexes in the ETC?

A

IV

40
Q

Complex I pumps how many H+?

A

4

41
Q

Complex II pumps how many H+?

A

none

42
Q

Complex III pumps how many H+?

A

4

43
Q

Q cycle

A

e- pass from CoQ to Cyt C reductase

cycle passes twice so 4H+ are pumped

44
Q

Cytochrome C is ______ peripheral protein and its binding to Complexes III and IV are electrostatic involving _____ residues

A

Mobile

Lysine

45
Q

Complex IV will pump how many H+?

A

2

46
Q

_____ contains Cu ions which are required for _____

A

Complex IV

O2 reduction to H2O

47
Q

Azide, cyanide and carbon monoxide bind to?

A

hene group in cyt C in complex IV

48
Q

One C ring rotation produces how many ATP at ATP synthase?

A

3 ATP

49
Q

Chemical/toxic inhibitors or complex I

A

Rotenone

50
Q

Chemical inhibitor of complex III

A

Antimycin

51
Q

Chemical inhibitor of complex IV

A

Hydrogen sulfide, cyanide and azide

52
Q

Carbon monoxide as an inhibitor

A

inhibits complex IV by binding to hemoglobin

It has a stronger affinity to Hg than O2

53
Q

Oligomycin

A

Binds to F0 in ATP synthase which inhibits H+ from entering

54
Q

Uncoupling proteins

A

Present in BAT which allow H+ to leak back into mitochondrial matrix which allows energy to be released as heat > non shivering thermogenesis

BAT is crucial in infants and promotes tolerance for cold temperature

55
Q

Synthetic uncouplers

A

2,4-dinitrophenol (2,4-DNP)

Aspirin (high doses)

56
Q

Most genetic abnormalities involivng the ETC are a results of mutations in _____

A

Mitochondrial DNA

Which mutates at a faster rate than nuclear DNA

57
Q

LHON

A

Lebers Hereditary Optic Neuropathy
Caused by a defect in NADH dehydrogenase
Leads to degeneration of the optic nerve and blindness

58
Q

MELAS

A

mitochondrial Myopathy, Encephalopathy, lactic Acidosis and Stroke

Caused by at least 10 different mutations in mitochondrial tRNA

59
Q

Leigh Syndrome

A

caused by mutations in mDNA and nuclear genes
Mutations in complexes I and IV and PDH complex

Damage is seen in the brain stem causing hypotonia, nystagmus and psychomotor regression, opthalmoplegia

60
Q

Products of TCA

A

2 CO2
3 NADH
1 FADH2
1 GTP

61
Q

B1 (TPP) deficiency affects

A

PDH Complex

alpha-ketoglutarate dehydrogenase complex

62
Q

TPP is a crucial coenzyme for the following enzymes:

A

Transketolase
PDH complex
alpha-ketoglutarate deyhdrogenase

63
Q

____ and _____ generate _____ as the first step in heme biosynthesis

A

Succinyl CoA and glycine

δ-ALA

64
Q

____ and ____ are competitive inhibitors on complex ____

A

CO and NO

IV

65
Q

____ are ____ noncompetitive inhibitors on complex ____

A

CN and H2S

IV

66
Q

Cyanide inhibits complex ____

A

IV, cytochrome oxidase

67
Q

Niacin is a precursor for _____

A

NADH

MCM - Molecular and Cellular Mechanisms) (6 decks)

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