the urea cycle Flashcards

1
Q

how many steps does it take to produce urea

A

5

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2
Q

where do the process that produce urea take place

A

mitochondrial matrix and cytosol of hepatocytes

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3
Q

what enters the cycle at step one

A

NH4+ (free ammonium)

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4
Q

what enters the cycle at step three

A

aspartate

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5
Q

step one occurs where

A

matrix of the mitochondria

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6
Q

step one consists of what

A

condensation of waste materials
HCO3- derived from CO2 produced in mitochondrial respiration

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7
Q

what increases the activity of step one

A

allosteric modulator, N-acetylglutamate

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8
Q

what allosterically activates step one and what is it used for

A

arginine, which is used to synthesis ornithine

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9
Q

where is step two

A

mitochondrial matrix

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10
Q

what happens during step two

A

carbamoyl phosphate donates carbamoyl group to ornithine to form citrulline

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11
Q

where is step three

A

cytosol

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12
Q

what happens during step three

A

a second amino group is introduced by condensation of citrulline and asparate via a citrullyl-AMP intermediate
produces argininosuccinate

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13
Q

what type of reactions are the first 3 reactions

A

condensation

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14
Q

where is step four

A

cytosol

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15
Q

where is step five

A

cytosol

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16
Q

what is hyperammonaemia

A

a result of impaired liver function

17
Q

what accompanies hyperammonaemia

A

raised levels of glutamine in blood as brain attempts to export excess ammonia

18
Q

treatment for hyperammonaemia

A

dialysis or benzoate therapy

19
Q

what happens in a hypercatabolic states

A

negative nitrogen balance
skeleton muscle protein broken down to produce glucose
urea synthesis and secretion increased
liver energy requirement increased

20
Q

what is a hypercatabolic state

A

metabolic shutdown

21
Q

when is metabolic acidosis observed

A

hypercatabolic states and prolonged fasts