Urea Cycle Flashcards

1
Q

Transamination

A

process for removing nitrogen from amino acids, tissues can use the carbon skeleton of the aa as fuel and transport the nitrogen to the liver for conversion into urea

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2
Q

Where is nitrogen converted into urea?

A

in the liver

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3
Q

PLP (pyridoxal phosphate)

A

coenzyme, active form of vitamin B6

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4
Q

urea formation

A

proteins -> amino acids -> NH2 removed -> forms ammonia, liver converts to urea

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5
Q

Deamination

A

the removal of an amino group from an organism, particularly from an amino acid

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6
Q

during fast what will be broken down to produce energy?

A

muscle protein

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7
Q

the break down of muscle protein causes the generation of what?

A

nitrogenous waste

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8
Q

urea will be excreted through ….

A

urine

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9
Q

healthy adults are in nitrogen balance, which means.

A

amount of nitrogen taken in = amount of nitrogen excreted (mostly through urea)

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10
Q

the properly functioning liver has a great capacity for what?

A

forming urea

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11
Q

The greater the amount of ammonia produced =

A

The greater amount of urea converted

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12
Q

high levels of ammonia indicates a problem with what organ?

A

The liver

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13
Q

ammonia =

A

liver

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14
Q

urea =

A

kidney

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15
Q

urea will be secreted from what organ?

A

The kidneys

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16
Q

Reactive Oxygen Species (ROS)

A

due to oxygen having 2 single electrons in 2 different orbitals, both with the same spin, it is a biradical

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17
Q

2 electrons with the same spin cannot …..

A

easily oxidize the bonds in organic compounds because of “spin restriction” - enzyme is required

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18
Q

ROS are generated

A

naturally by cellular process

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19
Q

free radicals

A

those that can exist independently, can take other electrons from other compounds (usually H’s)

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20
Q

radicals formed in enzymatic reactions

A

intermediates that form stable’s products and do not go “free”

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21
Q

what will ROS initiate?

A

chain reactions that damage substances within the cell

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22
Q

Sources of ROS

A
  1. Mitochondrial electron transport chain produces ATP to run cellular processes, but accidentally forms superoxide
  2. Ionizing radiation (X-rays, radioactive chemicals)
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23
Q

Cytochrome P450 enzymes

A

metabolizes drugs, alcohol, and chemical toxins

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24
Q

job of cytochrome P450 enzymes

A

oxidize the drugs and other substrates to make them more soluble for excretion

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25
Q

Radical intermediates of Cytochrome P450 can

A

escape and become free radicals, causing cell damage

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26
Q

What damage can be caused by ROS

A
  • Protein damage
  • Mitochondrial damage
  • Membrane damage
  • DNA damage
  • Cell swelling
  • Increased Permeability
  • Massive influx of CA2+
  • Lipid Per-oxidation
  • Respiratory
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27
Q

DNA damage due to ROS

A

strand breaks or 1/20 different alterations that can lead to mutations

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28
Q

Is there anything that can help repair the DNA damage done by the ROS?

A

If the DNA repair systems can fix the alterations than yes, if missed mutations will accumulate

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29
Q

Endogenous sources of ROS

A

Inside the body (O2, H2O2, OH, NO, O2)

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30
Q

Reactive Nitrogen-Oxygen Species (RNOS)

A

free radicals that cause damage to cellular components: DNA, cell membranes, enzymes, electron-transport chain

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31
Q

RNOS compounds come from where?

A

Produced in the cell or can be from outside sources that will damage our cells

32
Q

Inflammation free radicals

A

destroy invading pathogen or clean up dead cells in damaged tissues, activated neutrophils

33
Q

Respiratory burst (oxidative burst)

A

occurs in activated neutrophils, consumes oxygen to create reactive substances that will kill phagocytosed bacteria

34
Q

Is inflammation helpful or harmful?

A

either. can lead to collateral damage to the surrounding tissues

35
Q

Cellular defences put in place to do what?

A

fights the effects of free radicals

36
Q

oxidative stress

A

a condition in which the production of oxidants and free radicals exceeds the body’s ability to handle them and prevent damage

37
Q

endogenous antioxidants (ex. superoxide dismutases (SOD)) and catalase

A

have different isoforms that exist in different parts of the cells

38
Q

glutatione peroxidase

A

function to remove peroxiding formed outside of peroxisomes

39
Q

metals found in nutrients in the diet

A

Cu, Zn, Mn, Fe, Se

40
Q

Fat soluble Vit E and Waters soluble Vit C are involved in

A

neutralizing free radicals

41
Q

Vit E

A

donate an electron to radicals

42
Q

Vit C

A

donate an electron to Vit E to regenerate its antioxidant capacity

43
Q

Aged-related macular degeneration (AMD) is

A

A disease that blurs central vision, oxidative damage thought to be responsible for advancing the disease

44
Q

Where does the urea cycle exclusively take place?

A

in the liver

45
Q

When does nitrogen occur?

A

at physiological pH in the form of NH4 and ammonium in the blood

46
Q

Is urea toxic to the body? what happens to it?

A

No it is sent to the kidney and excreted from there

47
Q

What happens to the protein that is broken down and not used for protein synthesis?

A

deaminated

48
Q

TOO much protein means

A
  • cannot store protein
  • always functional
  • we turn it into glucose or fat to store
49
Q

When we have more protein what happens?

A
  • turns into glucose

- restores glycogen and the rest is stored as fat packed in VLDL

50
Q

What must nitrogen be converted into to be excreted in the urine and where?

A

turned into urea in the liver

51
Q

How can essential amino acids be made?

A

transamination

52
Q

What is PLP involved in?

A

amino acid metabolism

53
Q

Great amount of ammonia produced means

A

greater amount converted to urea

54
Q

When we create nitrogen through deamination what else occurs?w

A

we create ammonia which is toxic to CNS

55
Q

What happens to the ammonia created from deamination?

A

the liver will take it to create urea and send it to kidney to be excreted

56
Q

Elevated ammonia means

A

liver problem; chronic alcohol abuse, enzymes, hepatitis

57
Q

Elevated urea means

A

made it through the urea cycle; kidneys might be at risk

58
Q

Where do most of the steps in converting urea locate?

A

cytosol

59
Q

What is the most common X linked issue within the urea cycle?

A

orithine transcarbamoylase

60
Q

What causes alot of chronic diseases? and what are they?

A

reactive oxygen species

- cancer, respiratory problems, heart disease

61
Q

How are ROS generated?

A

by natural processes; unfortunate

62
Q

Source of ROS

A

the mitochondrial electron transport chain produces ATP to run cellular processes but accidentally forms superoxide

63
Q

What is an effective way to prevent DNA mutation when ROS occur?

A

base excision repair system

64
Q

What are RNOS

A

Exogenous forms of ROS

65
Q

Where do RNOS occur from?

A

those who smoke, poor diet etc

66
Q

RNOS damage can go to cellular components such as

A

DNA, cell membrane, enzymes, the ETC etc

67
Q

Endogenous

A

within our body

68
Q

Exogenous and antioxidant

A

taken into our body

69
Q

What can help reduce ROS?

A

vegetables and fruits

70
Q

Superoxidase dismutase is what

A

an enzyme in a number of different forms within a number of different cells

71
Q

When there is more LDL and no antioxidants to neutralize them what occurs?

A

atherosclerosis

72
Q

What vitamins are involved in neutralizing free radicals?

A

Vitamin E and C

73
Q

What does vitamin E do?

A

donate electron to free radicals

74
Q

Vitamin C can?

A

donate electron to vitamin E to regenerate its antioxidant capacity

75
Q

Carotenoids

A

derived from the diet and are antioxidant as well