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Topnotch Biochemistry (Sept 2017) > Nitrogen Metabolism > Flashcards

Nitrogen Metabolism Flashcards

1
Q

First phase of amino acid catabolism

A

Deamination

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2
Q

Steps in Deamination

A
  1. Transamination

2. Oxidative deamination

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3
Q

Enzyme used in transamination

A

Amino transferase

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4
Q

(3) Amino acids that don’t undergo transamination

A

Lysine
Threonine
Proline

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5
Q

Coenzyme in transamination

A

Pyridoxal phosphate (B6)

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6
Q

Enzyme used in oxidative deamination

A

Glutamate dehydrogenase

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7
Q

Enzyme that release ammonia from glutamine

A

Glutaminase

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8
Q

Where does urea cycle occurs?

A

Liver cytosol and mitochondria

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9
Q

(3) substrates of Urea cycle

A

NH3
Aspartate
CO2

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10
Q

Rate limiting step and enzyme in Urea Cycle.

A

NH3 → Carbamoyl phosphate

Carbamoyl phosphate synthetase I

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11
Q

What are the two enzymes used in mitcochondrial reactions in Urea cycle?

A
  1. CPS I

2. Ornithine transcarbomylase

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12
Q

2 Purely ketogenic AA

A

Lysine

Leucine

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13
Q

4 Glucogenic and Ketogenic AA

A

Trypthophan
Phenylalanine
Isoleucine
Tyrosine

(Try Philippine Islands, Ty)

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14
Q

Described with musty body oder.

Dx and enzyme deficient

A

PKU

Phenylalanine hydroxylase

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15
Q

Described with ochronosis, osler’s sign.

Dx and enzyme deficient

A

Alkaptonuria

Homogentisate oxidase

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16
Q

Described with stroke in the young, ectopia lentis, mental retardation. High plasma methionine

Dx and enzyme deficient

A

Homocystinuria

Cystathionine B-Synthase

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17
Q

Described with formation of kidney stones and staghorn calculi

Dx and 4 amino acids involved

A

Cystinuria

Cysteine, Ornithine, Lysine, Arginine
COLA

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18
Q

Enzyme deficient in Maple syrup urine disease

A

A-ketoacid dehydrogenase

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19
Q

4 steps in Heme synthesis

A
  1. Formation of delta-Aminolevulinic acid
  2. Formation of porphobilinogen
  3. Formation of uroporphyrinogen
  4. Formation of heme
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20
Q

Rate limiting step and enzyme of Heme synthesis

A

Formation of delta-Aminolevulinic acid

ALA synthase

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21
Q

Step inhibited by heavy metals (Lead) in Heme synthesis.

A

Formation of porphobilinogen and Formation of Heme

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22
Q

Enzyme used in formation of bilirubin diglucoronide

A

Bilirubin glucuronyl-transferase

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23
Q

Colorless bile

A

Urobilinogen

24
Q

Yellow bile

A

Urobilin

25
Q

Red orange bile

A

Bilirubin

26
Q

Green bile

A

Biliverdin

27
Q

(2) enzymes inactivated by Lead at heme synthesis

A

ALA synthethase and Ferrochelatase

28
Q

Used to measure bilirubin in serum

A

Van den Bergh Reaction

(-) methanol = direct bilirubin
(+) methanol = total bilirubin

29
Q

Gilber Syndrome

Direct or Indirect Hyperbilirubinemia

A

Indirect Hyperbilirubinemia

30
Q

Rotor Syndrome

Direct or Indirect Hyperbilirubinemia

A

Direct Hyperbilirubinemia

31
Q

Toxic Hyperbilirubinemia

Direct or Indirect Hyperbilirubinemia

A

Indirect Hyperbilirubinemia

32
Q

Hemolytic Anemias

Direct or Indirect Hyperbilirubinemia

A

Indirect Hyperbilirubinemia

33
Q

Crigler-Najjar Syndrome I

Direct or Indirect Hyperbilirubinemia

A

Indirect Hyperbilirubinemia

34
Q

Crigler-Najjar Syndrome II

Direct or Indirect Hyperbilirubinemia

A

Indirect Hyperbilirubinemia

35
Q

Dubin Johnson

Direct or Indirect Hyperbilirubinemia

A

Direct Hyperbilirubinemia

36
Q

Principal site of metabolism of branched chain AA

A

Skeletal muscle

37
Q

Glycogenesis

Active during Absorptive State or Post-Absorptive State

A

Absorptive

38
Q

Glycogenolysis

Active during Absorptive State or Post-Absorptive State

A

Post Absorptive

39
Q

Protein synthesis

Active during Absorptive State or Post-Absorptive State

A

Absorptive

40
Q

TAG synthesis

Active during Absorptive State or Post-Absorptive State

A

Absorptive

41
Q

Pentose Phosphate Pathway

Active during Absorptive State or Post-Absorptive State

A

Absorptive

42
Q

VLDL synthesis

Active during Absorptive State or Post-Absorptive State

A

Absorptive

43
Q

Gluconeogenesis

Active during Absorptive State or Post-Absorptive State

A

Post Absorptive

44
Q

Decrease Insulin:Glucagon Ratio

Active during Absorptive State or Post-Absorptive State

A

Post Absorptive

45
Q

Increase Insulin: Glucagon Ratio

Active during Absorptive State or Post-Absorptive State

A

Absorptive

46
Q

Ketogenesis

Active during Absorptive State or Post-Absorptive State

A

Post Absorptive

47
Q

Ketolysis

Active during Absorptive State or Post-Absorptive State

A

None

48
Q

Activates Carbamoyl Phosphate Synthase I

A

N-Acetyl Glutamate

49
Q

Enzyme deficient in Hyperammonemia type I

A

Carbamoyl Phosphate Synthase

50
Q

Enzyme deficient in Hyperammonemia type II

A

Ornithine transcarbamylase

51
Q

Enzyme deficient in Citrullinemia

A

Arginosuccinate synthetase

52
Q

Enzyme deficient in Argininosuccinate aciduria

A

Arginosuccinase

53
Q

Enzyme deficient in Arginemia

A

Arginase

54
Q

Most common deficiency involving urea cycle enzymes

A

Ornithine transcarbamylase deficiency

55
Q

Triad of Alkaptonuria

A

Ochronosis
Black Urine
Arthritis

56
Q

Pellagra skin rash, ataxia, neurodegenerative changes with defect in intestinal and renal transport of Tryptophan

A

Hartnup disease

57
Q

Also called Gammaglutamylcysteinylglycine

A

Gluthathione

Topnotch Biochemistry (Sept 2017) (9 decks)

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