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Biology and Biochem > Amino Acid Metabolism > Flashcards

Amino Acid Metabolism Flashcards

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1
Q

Alkaptonuria symptoms

A
  1. Dark urine
  2. Ochronosis
  3. Arthritis
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2
Q

Propionyl-CoA carboxylase converts what

A

Propionyl-CoA to Methylmalonyl-CoA

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3
Q

orphyria cutanea tarda genetics

A

AD

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4
Q

Carbamoyl phosphate synthetase 1 converts

A

NH4 + HCO3 + 2ATP to Carbamoyl phosphate

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5
Q

Uroporphyringoen decarboxylase converts

A

Uroporphyringoen-3 to Coproporphyrinogen 3

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6
Q

Homocystinuria symptoms

A
  1. DVT
  2. Stroke
  3. Atherosclerosis
  4. Marfan-like
  5. Mental retardation
  6. Joint contractures
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7
Q

Acute intermitten porphyria is due to a deficiency of

A

Porphobilinogen deaminase (Hydroxymethylbilane synthase)

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8
Q

Cystathionine Synthase converts what in AA metabolism

A

Homocysteine to Cystathionine

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9
Q

ALA synthase repressed by

A

Heme

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10
Q

Ornithine transcarbamoylase converts

A

Carbamoyl phosphate to Citrulline with addition of ornithine

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11
Q

Carbon Skeleton can be converted in the liver to

A

Glucose (glucogenic amino acids)

Acetyl CoA

Ketone bodies (Ketogenic amino acids)

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12
Q

Homocysteine methyl transferase converts what

A

Homocystein to methionine

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13
Q

Phenylalanine hydroxylase requires

A

Tetrahydrobiopterin

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14
Q

Lab results in a defect in the urea cycle

A

Hyperammonemia

Elevated blood glutamine

Decrease BUN

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15
Q

Aminotransferases convert

A

Amino acids to α-Keto acids

α-Ketoglutarate to Glutamate

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16
Q

Maple syrup urine disease symptoms

A
  1. Urine has odor of maple syrup
  2. Mental retardation
  3. Abnormal Muscle tone
  4. Ketosis
  5. Coma, death
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17
Q

PKU symptoms

A
  1. Mental retardation
  2. Musty odor
  3. Microcephaly
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18
Q

Porphobilinogen deaminase (Hydroxymethylbilane synthase) converts

A

Porphobilinogen to Hydroxymethylbilane

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19
Q

Amino groups released by deamination reaction form

A

Ammonium ions (NH4+)

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20
Q

Maple syrup urine disease is due to

A

Deficiency of Branched-chain ketoacid dehydrogenase

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21
Q

Urea synthesized from

A

Aspartate and carbamoyl phosphate

22
Q

Ferrochelatase inhibited by

A

Lead

23
Q

Alkaptonuria is due to

A

Deficiency of Homogentisate oxidase

24
Q

Alanine aminotransferase requires

A

B6

25
Q

Cystathionine synthase requires what

A

B6

26
Q

Ferrochelatase converts

A

Protoporphyrin 9 to Heme

27
Q

What Vit deficiency causes homocystinemia

A

Folate

Vit B12

Bit B6

28
Q

Aminotransferases require

A

B6

29
Q

Glutamine is

A

Ammonia attached to the γ-carboxyl group of glutamate

30
Q

Propionyl-CoA carboxylase requires

A

Biotin

31
Q

Homogentisate oxidase converts

A

Homogentisic acid to Maleylacetoacetate

32
Q

Carbamoyl phosphate synthetase 1 requires

A

N-acetylglutamate

33
Q

Porphyria cutanea tarda is due to deficiency of

A

Uroporphyringoen decarboxylase

34
Q

ALA dehydratase converts

A

ALA to Porphobilinogen

35
Q

Phenylalanine hydroxylase converts

A

Phenylalanine to Tyrosine

36
Q

Porphyria cutanea tarda symptoms

A
  1. Photosensitivity
  2. Inflammation, blistering, shearing of skin
  3. Hyperpigmentation
  4. Exacerbated by alcohol
  5. Red-brown to Deep-red urine
37
Q

Homocysteine methyl transferase require what

A

THF

B12

38
Q

Never give barbs in

A

Acute intermitten porphyria

39
Q

Carbamoyl Phosphate Synthetase deficiency characteristics

A
  • Hyperammoniemia
  • Increase blood glutamine
  • Decreased BUN
  • NO orotic aciduria
  • AR
  • Cerbral edema
  • Lethargy, convulsions, coma, death
40
Q

ALA dehydratase inhibited by

A

Lead

41
Q

Glutamate dehydrogenase requires

A

NAD

42
Q

Acute intermitten porphyria symptoms

A
  1. Acute abdominal pain
  2. Anxiety, Confusion, Paranoia
  3. Port-wine urine
43
Q

Glutaminase converts

A

Glutamine to Glutamate releasing NH3

44
Q

Methylmalonyl-CoA mutase converts what

A

Methylmalonyl-CoA to Succinyl-CoA

45
Q

Ornithine Transcarbamoylase deficiency characteristics

A
  • Hyperammonemia
  • Increased blood glutamine
  • Decreased BUN
  • Orotic aciduria
  • X-linked recessive
  • Cerebral edema
  • Lethargy, convulsions, coma, death
46
Q

Methylmalonyl-CoA mutase requires

A

B12

47
Q

ALA synthase requires

A

B6

48
Q

Acute intermittent porphyria genetics

A

AD

49
Q

Phenylketonuria is due to

A

Phenylalanine hydroxylase deficiency

50
Q

Homocystinuria is due to deficiency of what enzyme

A

Cystathionine synthase

51
Q

ALA synthase converts

A

Glycine + Succinyl-CoA to ALA

Biology and Biochem (15 decks)

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