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CPR > Nitrogen Metabolism > Flashcards

Nitrogen Metabolism Flashcards

1
Q

What is the major source of Nitrogen?

A

Protein

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2
Q

What are the major nitrogen excretory compounds? (5x)

A

1) urea
2) Ammonia
3) Creatinine
4) uric Acid (purine breakdown product)
5) urobilinogen (heme metabolism)

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3
Q

All excess amino acids are converted to ____

A

Glutamate

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4
Q

What is the main metabolic byproduct in extrahepatic tissues (particularly the brain)

A

Glutamine

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5
Q

What is the most prevalent nitrogen transporter in the blood?

A

Glutamine

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6
Q

What is the amino acid transporter of nitrogen from muscle?

A

Alanine

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7
Q

How is glutamate converted to Ammonia?

A

Glutamate Dehydrogenase

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8
Q

What reaction does Glutamate Dehydrogenase do?

A

Oxidative Deamination

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9
Q

What does Oxidative Deamination form?

A

a-keto acid

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10
Q

What enzyme conversts Ammonium to form Carbamoyl phosphate?

A

CPSaseI

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11
Q

Carbamoyl phosphate feeds into the ____

A

Urea cycle

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12
Q

Hartnup disease is a disease that affects reabsorption and absorption of

A

Nonpolar AA

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13
Q

Cystinuria disease is a disease that affects reabsorption and absorption of

A

Dibasic AA

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14
Q

If you have a mutation for Cystinuria and Hartnup Disease what organ systems are affected?

A

Renal

Small Intestines

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15
Q

Where are the transporters located on the PCT that uptake amino acids

A

Apical Membrane

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16
Q

What is the particular amino acid that isn’t transported in Hartnup?
What are the Amino acids that are of concern in Cystinuria?

A

1) Tryptophan

2) Cystine, Lysine, Arginine and Ornithine

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17
Q

Trp is a precursor for ___ ___ ___

A

Serotonin
Melatonin
Niacin

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18
Q

When a patient has HArtnup they will also have a ___ deficiency

A

Niacin

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19
Q

Manifestations of Hartnup: ___ ___ ___

A

1) Cerebellar Ataxia
2) Pellagra - like skin rash
3) Mental Disturbances

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20
Q

What is the treatment of Hartnup Disease: ___ _____

A

NA: Nicotinic Acid supplementation or Nicotinamide

High Protein Diet

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21
Q

IF a patient has cystinuria they will present with ____

A

Renal Colic

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22
Q

Treatment for Cystinuria

A

Dietary changes so that they are more alkaline

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23
Q

What enzyme def. causes Secondary PKU

A

Dihydrobiopterin Reductase

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24
Q

What causes classical PKU

A

Phenylalanine Hydroxylase def

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25
Q

A consequence of PKU is build up of Phenylalanine. Excessive Phenylalanine leads to ____ ____ ____.

A

1) Phenylpyruvate
2) Phenyllactate
3) Phenylacetate

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26
Q

Phenyllactate Causes ___ odor in urine

A

Musty

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27
Q

Treatment classical PKU

A

Dietary Supplement of Tyrosine and absence of phe in diet

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28
Q

How do you treat secondary PKU

A

Supplement with Tetrahydrobiopterin

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29
Q

What is the name of that test that new borns have to have to test for PKU

A

Post-parturition Guthrie Test

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30
Q

Diagnosis and initiation of dietary treatment of classic PKU must occur before the child is ___

A

2 weeks old

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31
Q

If Dihydrobiopterin is def. then there will be a like of which cofactor needed for tyrosine production.

A

THB (BH4)

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32
Q

With an absence of THB, patients will also present with neurological symptoms. Why?

A

Monoamine neurotransmitters production is disrupted

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33
Q

Phenyllactate and Phenylacetate will disrupt ____ and ___ transport to brain and ___ formation

A

Neurotransmission
Amino Acid
Myelin

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34
Q

Tyrosinemias are charcterized by _____ levels of tyrosine in blood

A

elevated

35
Q

Transient tyrosinemia in newborns is due to delayed expression of ____ necessary for Tyr catabolism.

A

4-hydroxyphenyl-pyruvate dioxygenase

36
Q

Treatment for Tyrosinemias

A

Dietary Restrictions of Phe and Tyr

37
Q

How can you differentiate Tyrosinemia from PKU in newborns

A

Hypertyrosinemia

38
Q

Type I Tyrosinemia is a defect in _______ and results in hereditary tyrosinemia.

A

Fumarylacetoacetate Hydrolase

39
Q

Infants with type I tyrosinemia will have a _____ like smell and develop severe liver failure due to build up of _____.

A

1) Cabbage

2) Succinylacetone

40
Q

Succinylacetone interferes the _____. What does it inhibit

A

1) TCA

2) Prophobilinogen Synthetase

41
Q

What is the treatment for type I tyrosinemia?

A

Nitisinone

42
Q

What does Nitisinone inhibit

A

4-HPPD

43
Q

Type II Tyrosinemia is due to a defect in _____

A

Tyrosine Aminotransferase

44
Q

Alkaptonuria is due to ____ def

A

Homogentisate Oxidase

45
Q

Homogentisate Oxidase is involved in _____ degradation pathway

A

Tyr

46
Q

Black Pigmentation in the intervertebral disks along with homogentisic aciduria, ochronosis, and arthritis is seen in ____

A

Alkaptonuria

47
Q

What is the triad in Alkaptonuria?

A

Homogentisic Aciduria
Ochronosis
Arthritis

48
Q

Type III Tyrosinemia is due to def. of

A

Hydroxyphenylpyruvate dioxygenase (4-HPPD)

49
Q

Type II Tyrosinemia presents with ___ and ___ hyperkeratosis, ___, and ___

A

1_ Palmar and Plantar

2) Corneal Lesions
3) intellectual Disability

50
Q

Ammonia Toxicity causes a reversal of ______ causeing the production of Glutamate instead of Alpha ketoglutarate

A

Glutamate Dehydrogenase

51
Q

Ammonia Toxicity causes depletion of what intermediates?

A

Alpha-ketoglutarate

Glutamate

52
Q

Ammonia Toxicity leads to decreased ____ since it causes the disruption of the TCA cycle

A

ATP

53
Q

What Enzyme produces glutamine from glutamate

A

Glutamine synthase

54
Q

With ammonia toxicity, Postsynaptic excitatory proteins are inhibited which _____ CNS function

A

Depresses

55
Q

Gout is caused by _____

A

High levels of uric acid in the blood

56
Q

Primary Hyperuricemia is caused by

A

Overproduction of uric acid

57
Q

Secondary Hyperuricemia is cause by

A

Underexcretion of uric acids

58
Q

What type of deposits are seen in gout

A

Sodium urate

59
Q

What types of diets should people avoid when dealing with Gout

A

1) Diets rich in purines
- Beans
- Spinach
- Lentils
2) Alcohol
3) Meat
4) Seafood

60
Q

Treatment of gout

A

Colchicine and Allopurinol

61
Q

What does Colchicine prevent?

A

Movement of granulocytes to affected areas

62
Q

What does Allopurinol inhibit

A

Xanathine Oxidase

63
Q

What accounts for most causes of hyperuricemia

A

Underexcretion

64
Q

What is the end product of purine metabolism

A

Uric Acid

65
Q

Patients with Acidosis will have decreaased ______ of uric acid

A

Tubular Secretion

66
Q

What is the rate limiting step of Urea Cycle

A

Carbomoyl Phosphate Synthetase (rate-limiting)

67
Q

What promotes the rate limiting step of Urea Cycle

A

NAG

68
Q

NAG is synthesize via what two reactants

A

Glutamate and Acetyl-coa

69
Q

Decreased NAG synthase results in

A

Hyperammonemia

70
Q

Symptoms of NAG synthase Deficiency

A

Vomiting
Refusal to Eat
Progressive Lethargy
Coma

71
Q

Orotic Aciduria occurs without hyperammonemima and BUN levels when there is a def. of

A

UMP Synthase

72
Q

Ornithine Transcarbamoylase Def. is _____ type of inheritance and presents with ___.

A

1) X-Linked Recessive

2) Orotic Aciduria w/ Hyperammonemia

73
Q

List the 6 enzymes that could be defective and cause hyperammonemia

A

1) NAGS
2) Carbamoyl Phosphate Synthetase I
3) Ornithine Transcarbamoylase
4) Argininosuccinate Lyase
5) Arginase

74
Q

CPSaseI is seen in what metabolic pathway

A

Urea Cycle

75
Q

CPSase II is seen in what metabolic pathway

A

Pyrimidine Synthesis

76
Q

CPSaseI is found in the ____

A

Mitochondria

77
Q

CPSaseII is found in the

A

Cytosol

78
Q

NAG activates

A

CPSaseI

79
Q

PRPP-activated activates

A

CPSaseII

80
Q

Without UDP Glucuronyl Transferase there will be an increase of

A

Unconjugated Bilirubin

81
Q

What enzyme congugates bilirubin

A

UGT

82
Q

What causes Gray Baby Syndrome which is due to Chloramphenicol Toxicity

A

UGT deficiency

83
Q

Symptoms of Gray Baby Syndrome

A

Circulatory Collapse due to impaired myocardial contractility

CPR (18 decks)

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