Final PP 1 Flashcards

1
Q

What is needed for the transport of FA from the cytoplasm to mitochondria?

A

ATP
Carnitine
Co-A

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2
Q

Write down the enzymes of urea cycle+localization

A
Carbomyl -p synthase (mitochondria)
Ornithine carbomyl transferase (mitochondria)
Argino-succinate synthase (cytosol)
Argino-succinate lyase (cytosol)
Arginase (cytosol)
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3
Q

LCAT action?

A

Catalyze esterification of cholesterol to form cholesterol ester

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4
Q

What is the reaction catalyzed by Glu DH?

A

Glu + NAD+ +H2O -> NH4 + a-KG +NADH +H

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5
Q

Give examples for enzymes that are activated upon phosphorylation

A

Glycogen phosphorylase

Phosphorylase kinase

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6
Q

What AA can be converted to serine in a single reaction?

A

Glycine

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7
Q

When does -plasma level of unesterified FA is increased?

A

Starvation
By glucagon
By ACTH

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8
Q

Localization and action of Acyl co-A DH?

A

Mitochondria

Trans double bond

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9
Q

What takes part in intestinal lipid digestion?

A

Pancreatic Lipase and bile acids

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10
Q

Human genetic PKU can result in:

A

Inability to convert Phe to Tyr

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11
Q

Write down the reaction catalyzed by Adenosine Deaminase

A

Adenosine +H2O -> Inosine +NH3

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12
Q

Action of ABCA1?

A

Efflux of cholesterol from the cell

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13
Q

What is the enzyme that catalyze

Phe -> Tyr

A

Phe Hydroxylase

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14
Q

What is the activity of Lipoprotein Lipase?

A

Hydrolysis of TAG of plasma Lipoproteins to supply FA to the tissues

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15
Q

Von Gierke disease is a result of the deficiency in

A

G-6-Pase

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16
Q

What is the role of Glutathione reductase in RBC?

A

It is needed for the removal of H2O2

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17
Q

What is the reaction catalyzed by HMG coA Reductase?

What is needed?

A

HMG coA -> Mevalonic acid

NADPH is needed for the reaction

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18
Q

What is the consequences of Acyl coA deficiency?

A

No ketone bodies synthesis

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19
Q

When do bile pigments are formed?

A

In the degradation of Heme

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20
Q
Orotic aciduria (inherited metabolic disease) when orotic acid is accumulated in the body
Occurs in which metabolic pathway?
A

Pyrimidine synthesis

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21
Q

What stimulates the transcription of ABCA1?

A

High intracellular conc of cholesterol

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22
Q

Where does B oxidation occurs?

A

Mitochondria

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23
Q

What apolipoprotein is an activator of LCAT?

A

Apo A-1

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24
Q

What is essential for the synthesis of FA?

A

CO2 and HCO3-

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25
Q

Lipoprotein Lipase activity?

A

Hydrolyses TAG from VLDL and chylomicrons

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26
Q

Enzymes of urea cycle are located in:

A

Mitochondria

Cytosol

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27
Q

Which aAA will enter the citric acid cycle as a-KG?

A

GAG PH

Gln
Arg
Glu
Pro
His
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28
Q

Substrates for ribonucleotide reductase?

A

ADP
GDP
CDP
UDP

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29
Q

Write down 3 inhibitors of Gluconeogenesis

A

F-2,6-BP
ADP
AMP

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30
Q

Which of this two will produce mitochondrial NADH?

Pyrimidine de novo synthesis
Purine de novo synthesis

A

Pyrimidine de novo synthesis

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31
Q

Enzyme converting inosine to hypoxanthine?

A

PNP (Purine Nucleoside Phosphorylase)

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32
Q

Which enzyme catalyzes the rate limiting step of cholesterol synthesis?

A

HMG coA Reductase

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33
Q

What happens to conjugated bilirubin in the serum during liver disease?

A

Decreased

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34
Q

What can we use as HMG coA Reductase inhibitors?

A

Statins

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35
Q

Which enzyme is allosterically modulated by malonyl co A?

Inhibition/activation?

A

Carnitine palmtoyl transferase 1

Inhibited

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36
Q

Where does the synthesis of NO starts from?

A

Arginine

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37
Q

What AA participate in the synthesis pf purine nucleotides?

A

Gln
Asp
Gly

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38
Q

How many subunits does PKC have?

A

1-single polypeptide chain

39
Q

How many units does PKA have?

40
Q

What receptor in the CNS function as Ca channel?

41
Q

What provides the methyl group here-

NE -> E

42
Q

What is Glutathione?

A

Tripeptide made of Glu, Cys, Gly

43
Q

What is the consequence of inherited Adenosine Deaminase deficiency?

A

Immunodeficiency

44
Q

What are the negative allosteric effectors of PFK1?

A

ATP
Citrate
H

45
Q

What is stimulated under high conc of F-2,6-Bi-P

A

Glycolysis

46
Q

Upon phosphorylation of PFK2 by PKA it is-

A

Inactivated, and the function of the FBPase is activated

47
Q

The glycogen branching enzyme catalyzes-

A

Formation of a1 -> 6 linkage

48
Q

What can induce gluconeogenesis in the kidney cortex?

A

Catecholamines

49
Q

What are the glucose transporters of the brain?

A

GLUT1 and 3

50
Q

Who has more mitochondria, astrocytes or neurons?

51
Q

What is the action of AMP-activated protein kinase in the muscle?

A

It phosphorylates acetyl co-A carboxylase and decreases malonyl co-A concentration in the muscle

52
Q

Describe the GABA shunt?

A

KG-Glu-GABA-succinate semi aldehyde-succinyl coA

53
Q

What activates tPA? (2)

A
  • Fibrin

- some of the plasmic fibrin degregation products stimulates it

54
Q

What provides precursor for the synthesis of nucleotides?

55
Q

Epinephrine triggers an increase rate of glycogen breakdown in muscle by causing-

A

Conversion of glycogen phosphorylase b to a

56
Q

Write down enzymes that needs Biotin as a prosthetic group (3)

A

Pyruvate carboxylase
Acetyl coA carboxylase
Propionyl coA carboxylase

57
Q

Following glycogenolysis, G1P is converted to

58
Q

Precursor for the biosynthesis of pyrimidine ring system includes:

A

Carbomyl phosphate and Asp

59
Q

LPL htdrolyze TAG from?

A

mature chylomicrons and VLDL

60
Q

Glu -> aKG is reductive deamination OR reductive amidation?

A

reductive amidation

61
Q

which proteins/enzymes participate in the reverse cholesterol transport?

A

Apo A1 and LCAT

62
Q

what is the consequence og mutation of the Ryanodine receptors?

A

malignant hyperthermia

63
Q

what are the characteristic of malignant hyperthermia?

A

rigidity of skeletal muscles

64
Q

which requires more than one step?

Ala->Pyruvate
Phe->Succinate
Asp->OAA
Glu->aKG
Pro->Glu
A

Pro->Glu

Phe->Succinate

65
Q

where do Heme Oxygenase plays a role?

A

in the degregation of Heme

66
Q

which of the next signaling components play a role in the action of glucagon?

  1. B receptor
  2. PKA
  3. cAMP
  4. Gs protein
  5. Gi protein
A
  1. PKA
  2. cAMP
  3. Gs protein

(B receptor is for NE)

67
Q

what are leptins?

A

polypeptides produced by the adipose tissue

68
Q

clinical symptoms of antiphospholipid syndrom can be treated wirh? (2)

A

vit K antagonist

Heparin

69
Q

what is the first step of fructose metabolism?

A

phosphor of fructose to F-1-P

70
Q

order this by ATP energy

Ala
palmitate
glucose

A

palmitate>glucose>Ala

71
Q

What is the subunit composition of the ganglion type nicotinic receptor?

72
Q

What activates Tyr hydroxylase?

73
Q

Put this in the right order of GABA shunt

GABA
Succinate semialdehyde
Glu
aKG
Succinyl co A
A

KG->Glu->GABA->succinate semialdehyde->succinyl co A

74
Q

Substrates for thrombin in blood coagulation?

A
Fibrinogen
V
VIII
XI
XIII 
Platelets
75
Q

What is the primary target of strophantin?

Other name?

A

Ouabain
Its a cardiac glycoside
Inhibits Na/K ATPase

76
Q

Write down the mechanism of action of Ouabain:

A

Na/K ATPase inhibition ->accumulation of intracellular Na -> reduced activity of Na/Ca exchanger -> elevation of intracellular Ca -> higher cardiac contractility

77
Q

Open probability of ion channel can range from -

78
Q

Phosphorylation decrease/increase the activity of CFTR Cl channel

79
Q

The gating charge in the voltage sensor of voltage gated K channels is provided by conserved _____ and _____ residues on the ____ helix

80
Q

Myasthenia gravis happens because-

A

Autoantibodies directed against the nicotinic acetyl choline receptor

81
Q

How is the formation of blood coagulation complexes inhibited in the clinic?

A

Vitamin K inhibitors

82
Q

What kind of molecule exerts irreversible inhibition of Ach esterase?

A

Organic phosphate

83
Q

What does phosphorylated CFTR Cl channel need for gating?

84
Q

The gate of the K channel id located close to the _________ side

A

Intracellular

85
Q

How does dopamine influences the activity of Na/K ATPase in the kidney?

86
Q

Name the metabolite that is formed in the brain in the metabolism of NE and can be detected in the urine

87
Q

What is the coenzyme for methylmalonyl mutase?

88
Q

Amino acids with 4 carbons?

89
Q

What happens to Vmax in the presence of competitive inhibitor?

A

Stay the same

90
Q

What happens to Vmax in the presence of non-competitive inhibitor?

91
Q

what does the deficiency in branched-chain α-ketoacid dehydrogenase complex causes?

A

Maple syrup disease

92
Q

two NADPH producing enzymes of the PPP?

A

G-6-P DH

6-phosphogluconate DH

93
Q

two pathways for arachidonic acid?

A

Cycloxygenase

Lipoxygenase (produces leukotriens)