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AAA Biochemistry > Nitrogen Metabolism > Flashcards

Nitrogen Metabolism Flashcards

1
Q

Sources of amino acid pool (3)

A

Turnover of body protein
Dietary intake
Synthesis of non-essential AA

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2
Q

2 main steps in removing nitrogen from AA

A

Transamination

Oxidative Deamination

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3
Q

AA transferred to a-ketoglutarate in transamination to form glutamate except: (2)

A

Lysine

Threonine

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4
Q

Transamination enzyme and co-enzyme?

A 
Aminotransferases; 
Pyridoxal phosphate (B6)
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5
Q

In transamination, pyruvate interconvert with?

A

Alanine

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6
Q

In transamination, aspartate interconvert with?

A

Oxaloacetate

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7
Q

Transamination vs oxidative deamination:
Occurs in all cell of the body
No free ammonia

A

Transamination

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8
Q

Transamination vs oxidative deamination:
Occurs in the liver and kidney?
Only for glutamate
Liberates ammonia

A

Oxidative deamination

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9
Q

Glutamate + Ammonia?

Enzyme?

A

Glutamine

Glutamine Synthetase

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10
Q

Glucose-Alanine Cycle
Pyruvate + Glutamate = ?
Enzyme?

A

Alanine aminotransferase (ALT/SGPT)

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11
Q

Deaminates glutamine to produce ammonium ion which is excreted from the body.

A

Glutaminase

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12
Q

Glutaminase is present where?

A

Small intestine

Kidney

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13
Q

Urea cycle is present only in:

A

Liver

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14
Q

Substrates of Urea Cycle (3)

A

NH3 from
Aspartate
CO2

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15
Q

Steps in Urea Cycle (8)

A 
Ornithine
Carbamoyl Phosphate
Citrulline
Aspartate
Arginosuccinate
Fumarate 
Arginine
Urea

(Old Colorful Cats Are Also Fussing About Urea)

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16
Q

Urea Cycle Enzymes:

Formation of carbamoyl phosphate

A

Carbamoyl phosphate synthetase I

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17
Q

Urea Cycle Enzymes:

Formation of citrulline

A

Ornithine transcarbamoyolase

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18
Q

Urea Cycle Enzymes:

Synthesis of Arginosuccinate

A

Arginosuccinate Synthetase

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19
Q

Urea Cycle Enzymes:

Cleavage of Arginosuccinate to form arginine

A

Argininosuccinase

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20
Q

Urea Cycle Enzymes:

Arginine cleavage to form urea and ornithine

A

Arginase

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21
Q

Urea Cycle Enzymes:

Rate Limiting Step and Enzyme?

A

CO2 + NH3 –> Carbamoyl phosphate

CPS I

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22
Q

Urea Cycle Enzymes:

Energy Requirement

A

4 moles of ATP

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23
Q

Urea Cycle Enzymes:

Cofactors

A

N-acetylglutamate

Biotin

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24
Q

Hereditary hyperammonemia

Type 1

A

Carbamoyl phosphate synthetase I

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25
Q

Hereditary hyperammonemia

Type 2

A

Ornithine transcarbamoylase

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26
Q

Hereditary hyperammonemia

Dx

A

Hyperammonemia
Inc blood glutamine
Decreased BUN

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27
Q

Hereditary hyperammonemia

Treatment

A

Low protein diet
Na Benzoate
Phenylpyruvate

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28
Q

Acquired hyperammonemia

Sx

A 
Tremors
Slurring of speech
Somnolence
Vomiting
Blurring of vision
Cerebral edema
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29
Q

Ketogenic AA

A

Leucine

Lysine

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30
Q

Ketogenic and Glucogenic

A

Phenylalanine
Tyrosine
Tryptophan
Isoleucine

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31
Q

Yields of ketogenic AA

A

Acetoacetate

Acetyl coA

32
Q

Yields of glucogenic AA

A

Glucose

Glycogen

33
Q 
Entry points:
Glutamine
Glutamate
Proline
Arginine
Histidine
A

Alpha ketoglutarate

34
Q 
Entry points:
Alanine
Serine
Glycine
Cysteine
Threonine
Tryptophan
A

Pyruvate

35
Q

Entry points:
Phenylalanine
Tyrosine

A

Fumarate

36
Q 
Entry points:
Methionine
Valine
Isoleucine
Threonine
A

Succinyl CoA

37
Q

Entry points:
Aspartate
Asparagine

A

Oxaloacetate

38
Q

Synthesis of Non Essential Amino Acids

Transamination of alpha ketoacids (3)

A

Alanine
Aspartate
Glutamate

39
Q

Synthesis of Non Essential Amino Acids

Amidation of glutamate and aspartate (2)

A

Glutamine and asparagine

40
Q

Synthesis of Non Essential Amino Acids

Synthesized from glutamate

A

Proline

41
Q

Synthesis of Non Essential Amino Acids

Made from methionine and serine

A

Cysteine

42
Q

Synthesis of Non Essential Amino Acids

Made from 3-phosphoglycerate

A

Serine

43
Q

Synthesis of Non Essential Amino Acids

Made from serine

A

Glycine

44
Q

Synthesis of Non Essential Amino Acids

Made from phenylalanine

A

Tyrosine

45
Q

Raw Material in Biosynthesis:

Heme, purines, creatine, bile acid conjugation

A

Glycine

46
Q

Raw Material in Biosynthesis:

Phospholipid & sphingolipid, purines, thymines

A

Serine

47
Q

Raw Material in Biosynthesis:

GABA

A

Glutamine

48
Q

Raw Material in Biosynthesis:
Thioethanolamine of CoA
Taurine

A

Cysteine

49
Q

Raw Material in Biosynthesis:

Histamine

A

Histidine

50
Q

Raw Material in Biosynthesis:

Creatinine, polyamine, NO

A

Arginine

51
Q 
Raw Material in Biosynthesis:
Serotonin
NAD
NADP
Melatonin
A

Tryptophan

52
Q

Raw Material in Biosynthesis:
Catecholamine
Thyroid hormones
Melanin

A

Tyrosine

53
Q

Deficiency in phenylketonuria

A

Decreased phenylalanine hydroxylase

Decreased tetrahydrobiopterine cofactor

54
Q

Tx in Phenylketonuria

A

Decreased phenylalanine

Increased tyrosine

55
Q

Deficiency in alkaptonuria

A

Homogentisic acid oxidase

56
Q

Black urine
Ochronosis
Debilitating arthralgia

A

Alkaptonuria

57
Q

Deficient tyrosinase or tyrosine transporters

A

Albinism

58
Q

Homocystinuria

Tx for: Cystathione synthase deficiency

A

Low methionine
High cysteine
High B6
High folate

59
Q 
Sx:
Mental retardation
Osteoporosis
Tall stature
Kyphosis
Lens subluxation
Atherosclerosis (Stroke in the Young)
A

Homocystinuria

60
Q

Common inherited defect of renal tubular amino acid transporter at PCT of kidneys

A

Cystinuria

61
Q

Cystinuria: transporter defect for what proteins?

A

Cystine
Ornithine
Lysine
Arginine

62
Q

Tx for cystinuria

A

Acetazolamide to alkalinaze urine

63
Q

Deficiency in MSUD

A

Alpha ketoacid dehydrogenase

64
Q

Heme Synthesis:

Rate limiting step?
Enzyme?
Cofactor?

A

Glycine + succinyl coA –> alpha-levulinic acid

ALA synthase

Pyridoxine

65
Q

Heme Synthesis:

Porphobilinogen formation

Enzyme?
How many ALA?
Cofactor?
Inhibited by?

A

ALA dehydratase
Cofactor: Zinc
Inhibited by: Heavy metal ions

66
Q

Heme Synthesis:

Heme formation
Iron is introduced to:
Rate enhanced by:
Inhibited by:

A

Heme formation
Iron is introduced to: Protoporphyrin IX
Rate enhanced by: Ferrochelatase
Inhibited by: Lead

67
Q

Photosensitivity
Abdominal pain
Neuropsychiatric symptoms
Most common porphyria

A

Porohyria cutanea tarda

68
Q

Pyridoxine deficiency with isoniazid therapy

A

Sideroblastic anemia with ringed sideroblasts

69
Q

Enzymed inactivated by lead poisoning

A

ALA Dehydratase

Ferrochelatase

70
Q

Disease with increased urinary ALA and free erytrocyte porphyrin

A

Lead poisoning

71
Q

Enzyme involved:
X-linked sideroblastic anemia

Sx: Anemia

A

ALA Synthase (erythroid)

72
Q

Acute intermittent porphyria

Abd pain
Neuropsych symptoms

Urinary porphobilinogen +
Uroporphyrin positive

A

Uroporphyrinogen I synthase

73
Q

Congenital erythropoietic

No photosensitivity

Porphobilinogen negative
Uroporphyrin positive

A

Uroporohyrinogen III synthase

74
Q

Heme –> biliverdin –> bilirubin

Enzyme?

A

Heme oxygenase

75
Q

Bilirubin conjugated to bilirubin diglucoronide

Enzyme?

A

Bilirubin diglucuronyltransferase

76
Q

Deficient in Crigler Najjar I, II, and Gilbert?

A

Bilirubin diglucuronyltransferase

77
Q

Rotor Syndrome

Dubin Johnson syndrome

A

Conjugated hyperbilirubinemia

AAA Biochemistry (7 decks)

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