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Nitrogen Metabolism Flashcards

(83 cards)

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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
A consequence of PKU is build up of Phenylalanine. Excessive Phenylalanine leads to ____ ____ ____.
1) Phenylpyruvate 2) Phenyllactate 3) Phenylacetate
26
Phenyllactate Causes ___ odor in urine
Musty
27
Treatment classical PKU
Dietary Supplement of Tyrosine and absence of phe in diet
28
How do you treat secondary PKU
Supplement with Tetrahydrobiopterin
29
What is the name of that test that new borns have to have to test for PKU
Post-parturition Guthrie Test
30
Diagnosis and initiation of dietary treatment of classic PKU must occur before the child is ___
2 weeks old
31
If Dihydrobiopterin is def. then there will be a like of which cofactor needed for tyrosine production.
THB (BH4)
32
With an absence of THB, patients will also present with neurological symptoms. Why?
Monoamine neurotransmitters production is disrupted
33
Phenyllactate and Phenylacetate will disrupt ____ and ___ transport to brain and ___ formation
Neurotransmission Amino Acid Myelin
34
Tyrosinemias are charcterized by _____ levels of tyrosine in blood
elevated
35
Transient tyrosinemia in newborns is due to delayed expression of ____ necessary for Tyr catabolism.
4-hydroxyphenyl-pyruvate dioxygenase
36
Treatment for Tyrosinemias
Dietary Restrictions of Phe and Tyr
37
How can you differentiate Tyrosinemia from PKU in newborns
Hypertyrosinemia
38
Type I Tyrosinemia is a defect in _______ and results in hereditary tyrosinemia.
Fumarylacetoacetate Hydrolase
39
Infants with type I tyrosinemia will have a _____ like smell and develop severe liver failure due to build up of _____.
1) Cabbage | 2) Succinylacetone
40
Succinylacetone interferes the _____. What does it inhibit
1) TCA | 2) Prophobilinogen Synthetase
41
What is the treatment for type I tyrosinemia?
Nitisinone
42
What does Nitisinone inhibit
4-HPPD
43
Type II Tyrosinemia is due to a defect in _____
Tyrosine Aminotransferase
44
Alkaptonuria is due to ____ def
Homogentisate Oxidase
45
Homogentisate Oxidase is involved in _____ degradation pathway
Tyr
46
Black Pigmentation in the intervertebral disks along with homogentisic aciduria, ochronosis, and arthritis is seen in ____
Alkaptonuria
47
What is the triad in Alkaptonuria?
Homogentisic Aciduria Ochronosis Arthritis
48
Type III Tyrosinemia is due to def. of
Hydroxyphenylpyruvate dioxygenase (4-HPPD)
49
Type II Tyrosinemia presents with ___ and ___ hyperkeratosis, ___, and ___
1_ Palmar and Plantar 2) Corneal Lesions 3) intellectual Disability
50
Ammonia Toxicity causes a reversal of ______ causeing the production of Glutamate instead of Alpha ketoglutarate
Glutamate Dehydrogenase
51
Ammonia Toxicity causes depletion of what intermediates?
Alpha-ketoglutarate | Glutamate
52
Ammonia Toxicity leads to decreased ____ since it causes the disruption of the TCA cycle
ATP
53
What Enzyme produces glutamine from glutamate
Glutamine synthase
54
With ammonia toxicity, Postsynaptic excitatory proteins are inhibited which _____ CNS function
Depresses
55
Gout is caused by _____
High levels of uric acid in the blood
56
Primary Hyperuricemia is caused by
Overproduction of uric acid
57
Secondary Hyperuricemia is cause by
Underexcretion of uric acids
58
What type of deposits are seen in gout
Sodium urate
59
What types of diets should people avoid when dealing with Gout
1) Diets rich in purines - Beans - Spinach - Lentils 2) Alcohol 3) Meat 4) Seafood
60
Treatment of gout
Colchicine and Allopurinol
61
What does Colchicine prevent?
Movement of granulocytes to affected areas
62
What does Allopurinol inhibit
Xanathine Oxidase
63
What accounts for most causes of hyperuricemia
Underexcretion
64
What is the end product of purine metabolism
Uric Acid
65
Patients with Acidosis will have decreaased ______ of uric acid
Tubular Secretion
66
What is the rate limiting step of Urea Cycle
Carbomoyl Phosphate Synthetase (rate-limiting)
67
What promotes the rate limiting step of Urea Cycle
NAG
68
NAG is synthesize via what two reactants
Glutamate and Acetyl-coa
69
Decreased NAG synthase results in
Hyperammonemia
70
Symptoms of NAG synthase Deficiency
Vomiting Refusal to Eat Progressive Lethargy Coma
71
Orotic Aciduria occurs without hyperammonemima and BUN levels when there is a def. of
UMP Synthase
72
Ornithine Transcarbamoylase Def. is _____ type of inheritance and presents with ___.
1) X-Linked Recessive | 2) Orotic Aciduria w/ Hyperammonemia
73
List the 6 enzymes that could be defective and cause hyperammonemia
1) NAGS 2) Carbamoyl Phosphate Synthetase I 3) Ornithine Transcarbamoylase 4) Argininosuccinate Lyase 5) Arginase
74
CPSaseI is seen in what metabolic pathway
Urea Cycle
75
CPSase II is seen in what metabolic pathway
Pyrimidine Synthesis
76
CPSaseI is found in the ____
Mitochondria
77
CPSaseII is found in the
Cytosol
78
NAG activates
CPSaseI
79
PRPP-activated activates
CPSaseII
80
Without UDP Glucuronyl Transferase there will be an increase of
Unconjugated Bilirubin
81
What enzyme congugates bilirubin
UGT
82
What causes Gray Baby Syndrome which is due to Chloramphenicol Toxicity
UGT deficiency
83
Symptoms of Gray Baby Syndrome
Circulatory Collapse due to impaired myocardial contractility

CPR (18 decks)

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