P3 BIOCHEMISTRY UREA CYCLE Flashcards

1
Q

Ammonia chemical characteristics

A

highly water soluble
colorless
irritant gas , pungent odor

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2
Q

Ammonia reacts with water forming

A

strong alkaline solution + ammonium hydroxide

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3
Q

sources of blood ammonia

A

Protein catabolism
Skeletal muscles
Intestinal bacteria

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4
Q

Protein catabolism is done by

A

liver

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5
Q

normal blood ammonia levels

A

10-50 micro mol/ L

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6
Q

Increased ammonia levels can indicates

A

severe liver defect

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7
Q

Ammonia levels can arise due to

A

Significant collateral circulation
Parenchymal cell dysfunction

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8
Q

major disposal form of ammonia

A

urea

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9
Q

site of urea cycle

A

Mitochondrial matrix
cytosol

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10
Q

how many ATP molecules are utilised during urea cycle

A

3 ATP compound

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11
Q

the enzyme that breaks down glutamate into ammonia is

A

Glutamate Dehydrogenase

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12
Q

Enzyme present in the liver that helps ls in the release of ammonia

A

Glutaminase

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13
Q

Carrier of ammonia from muscle to liver

A

Alanine

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14
Q

once ammonia is in the mitochondria of hepatocyte , it will combine with bicarbonate forming

A

Carbamoyl phosphate

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15
Q

Enzyme converting ammonia + HCO3 to carbamoyl phosphate

A

carbamyl phosphate synthase 1

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16
Q

ATP molecules utilised by CPS1

A

2 ATP molecules

17
Q

carbamoyl phosphate combined with what forming Citrulline

A

Ornithine

18
Q

Enzyme that converts carbamoyl phosphate to citrulline

A

ornithine transcarbamylase OTC

19
Q

Exiting into cytosol , citrulline combines with aspartate forming

A

argininosuccinate

20
Q

formation of argininosuccinate is done by which enzyme

A

argininosuccinate synthetase

21
Q

how many ATP molecules are utilised by AS enzyme

A

1

22
Q

argininosuccinate breaks down into

A

fumarate & arginine

23
Q

Arginine with enzyme ariginase forms

A

urea

24
Q

role of ammonia related to proteins

A

Anabolism & catabolism of amino acids

25
Q

other roles of ammonia

A
  • maintain acid base balance
  • conserve cations
26
Q

levels of ammonia in metabolic acidosis

A

Increased

27
Q

levels of ammonia in metabolic alkalosis

A

Decreased

28
Q

Ammonia toxicity is due to increase in what enzymes

A

glutamate dehydrogenase
Glutamine synthase

29
Q

Hyperactivity of these enzymes results in

A

Decreased alpha-keto-glutarate

30
Q

most sensitive organ to ammonia toxicity

A

brain

31
Q

Ammonia levels in hepatic failure

A

high

32
Q

Ammonia & glutamine levels in inherited disorders of urea cycle enzymes

A

high

33
Q

Substances that can be used to promote waste nitrogen excretion

A
  • sodium benzoate
  • sodium phenylacetate
34
Q

Sodium benzoate conjugates with

A

Glycine

35
Q

sodium phenylacetate conjugates with

A

glutamine

36
Q

Sodium benzoate + glycine forms

A

hippuric acid

37
Q

Sodium phenylacetate + glutamine forms

A

phenylacetylglutamine