Nitrogen Turnover Flashcards

1
Q

nitrogen compoounds are degraded into mostly

A
  • urea

- urea levels are the only thing that changes in excretion with regards to proteins intake

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2
Q

excretion in urine comes in four forms

A
  • ammonia
  • urea
  • uric acid (purine breakdown product)
  • creatinine (proportional to muscle mass)
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3
Q

balance of nitrogen in the body

A
  • intake from protein in the diet
  • goes into amino acid pool and can then be used for body protein or made into urea and excreted
  • if you are in a positive balance you will have more going to body protein than you are excreting
  • during times of insufficient protein, you have more going back into the amino acid pool from the body protein and that is being made into urea
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4
Q

tramua and urea balance

A
  • high energy requirement

- a lot more going into the amino acid pool and being made into urea

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5
Q

what are the 3 steps of removin nitrogen

A
  • transfer to a common carrier
  • ammonia is regenerated in the liver
  • ammonia is incoporated into urea
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6
Q

what is a common carrier of nitrogen

A

-alpha ketogluterate which then turns into glutamate

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7
Q

PLP

A
  • is used by aminotransferase in order to transfer a nitrogen from an amino acid a common carrier
  • arises from VB6
  • exhanges a keto group fro a amine group
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8
Q

glutamate dehydrogenase

  • location
  • function
  • what happens to its product in the liver
  • cofactors
A
  • this is located in the mitochondria and is fundamental for the release of ammonia so it can be acted on by CPSI to create carbamoyl phsophate for the urea cycle
  • in liver, the ammonia produced can be incorporated into urea
  • urea production is significant only in the liver
  • uses NAD or NADP as cofactors
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9
Q

regulation of glutamate dehydrogenase

A
  • activity increased by ADP and GDP

- activity decreased by ATP and GTP

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10
Q

what is ammonia produced from on asparagine and glutamine

A

-amide groups

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11
Q

minor way to dispose of ammonia

A
  • make glutamate by running the glutamate dehydrogenase reaction in reverse
  • this is when the urea cycle is saturated
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12
Q

major way of disposing of ammonia

A
  • make glutamine via the glutamine synthase reaction
  • used to fix ammonia especially in the brain; also occurs in muscle, lungs, and adipose tissue
  • glutamine is a majr carrier of n in the blood
  • glutamine levels increase after a protein rich meal
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13
Q

problem with hyperammonemia in children

A
  • glutamate can only hold so much ammonia as glutamine before it maxes out and the body runs out of glutamine
  • when this happens, hyperammonemia will present and this can cause serious health problem (especially in the brain)
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14
Q

urea cycle order and mnemonic

A
  • ordinarily careless crappers are also frivolous about urination
  • ornithing, carbamoyl phosphate, citrulline, aspartate, arginosuccinate, fumarate, arginine, urea
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15
Q

CPSI

A
  • founds in the mitochondria
  • needs NAG to function
  • NAG is made by NAG synthase using acetyl-CoA and glutamate
  • NAG synthase uses arginine
  • produces carbamoyl phosphate from NH4 which comes from the glutaminase and glutamate DH reactions
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16
Q

how is Nag synthase deficiency treated?

A

with carbamoyl glutamate

17
Q

what does a deficiency in any of the urea cycle enymes result in?

A

-elevated ammonia

18
Q

where do the two nitrogens that make up urea come from?

A

aspartate and NH4 from carbamoyl phosphate

19
Q

how many ATPs are used to make urea

A

4

20
Q

clinical symptoms of a inoperative or stressed urea cycle -

A
  • developmental delays
  • cognitive delays
  • seizure
  • protein intolerance
21
Q

diagnosing urea cycle defs

A
  • blood/urine amino acids analyses for ammonia

- enzyme assay

22
Q

treatment of yrea cycle disorders

A
  • restrict proteins from diet, this lowers nitrogen production
  • supplement with arginine
  • increase nitrogen excretion using benzoate or pheylacetate or phenylbutyrate
23
Q

how does the urea cycle interact with carb metabolism

A
  • argininosuccinate is converted into fumerate using argininosuccinase
  • fumerate can then be converted into OAA for gluconeogenesis, or into malate for pyruvate/PEP or sent to the mitochondria to directly aparticipate in the TCA
24
Q

why might we need to replenish ornithine or arginine and how do we do so

A

-ornithin can be used to make polyamine (spermine)
-arginine is used to make NO which is a vasodilator
-glutamine can be used to make glutamic semialdehyde which can be converted into ornithine
glutamic semialdehyde can also be made from ornithine which can then be used to make proline