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aDems Unit 2b- Lindsey's > The Urea Cycle and Specialized Products > Flashcards

The Urea Cycle and Specialized Products Flashcards

1
Q

What happens after a protein is polyubiquitinated?

A

it is degraded in the proteasome

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2
Q

Name the aa’s that are both glucogenic and ketogenic.

A
  1. Tyrosine
  2. Isoleucine
  3. Phenylalanine
  4. Tryptophan
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3
Q

What disease results when you cannot break down branched chain aa’s?

A

Maple Syrup Urine Disease (MSUD)

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4
Q

What enzyme converts arginine –> citrulline? What is the product?

A
  • NO synthase
  • NO
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5
Q

What does thyroid peroxidase do?

A

it oxidizes iodide (I-) to I2

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6
Q

What vitamin is essential for gamma-glutanyl carboxylase function?

A

vitamin K

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7
Q

What does it mean to be a glucogenic aa?

A
  • it produces pyruvate, fumarate, or other Kreb Cycle intermediates
  • a carbon source can be derived from it
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8
Q

What does muscle use to transport ammonia?

A

alanine

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9
Q

Which enzyme converts argininosuccinate –> arginine? In which cycle?

A
  • arginosuccinate lyase
  • the urea cycle
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10
Q

What transports ammonia thru the blood?

A

glutamine

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11
Q

What does it mean to be a ketogenic aa?

A

no net production of glucose (only acetyl-CoA or acetoacetate)

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12
Q

In the _____, bilirubin diglucuronide (conjugated bilirubin) is oxidized to stercobilin.

A

intestine

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13
Q

What is the function of thyroxin binding globulin (TBG)?

A

to transport T4/T3

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14
Q

Which enzyme converts arginine –> ornithine + urea? In which cycle?

A
  • arginase
  • in the urea cycle
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15
Q

What enzyme is vitamin C essential for?

A

for the hydroxyproline and hydroxylysine hydroxylation reactions

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16
Q

In the intestine, bilirubin diglucuronide (conjugated bilirubin) is oxidized to ______.

A

stercobilin

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17
Q

____ stimulates iodide uptake and stimulates release of T4/T3.

A

Thyroid stimulating hormone (TSH)

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18
Q

_____ occurs when branched-chain-a-ketoacid dehydrogenase complex is deficient, and there is consequently a buildup of the a-keto acids in urine.

A

Maple Syrup Urine Disease (MSUD)

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19
Q

What does arginosuccinate synthase do? In which cycle?

A
  • converts citrulline + aspartate –> argininosuccinate
  • in the urea cycle
20
Q

_____ is used to make T4 that is then converted to T3.

A

Tyrosine

21
Q

What does arginase do? In which cycle?

A
  • converts arginine –> ornithine + urea
  • in the urea cycle
22
Q

What happens with a vitamin C deficiency?

A

scurvy- decreased collagen strength, decreased RBCs, easy bruising and hemorrhages

23
Q

______ such as heme are cyclic molecules made of 4x pyroles primarily produced in the liver.

A

Porphyrins

24
Q

Where is carbamoyl phosphatase synthetase I found?

A

the mitochondria

25
Q

In the _____, bilirubin is conjugated with glucuronic acid.

A

liver

26
Q

What enzyme is vitamin B6 required for?

A

aminotransferase

27
Q

What oxidizes iodide (I-) to I2?

A

thyroid peroxidase

28
Q

______ contains Tyr residues iodinated to form T4/T3.

A

Thyroglobulin (Tg)

29
Q

In the liver, bilirubin is conjugated with ______.

A

glucuronic acid

30
Q

What binds to porphyrins?

A

Fe2+

31
Q

Name an allosteric activator of carbamoyl phosphatase synthetase I.

A

N-acetylglutamate

32
Q

This transports T4/T3.

A

thyroxin binding globulin (TBG)

33
Q

____ inhibits 2 enzymes in porphyrin synthesis leading to poisoning.

A

Lead

34
Q

_________ is the general term for diseases in porphyrin synthesis.

A

Porphyrias

35
Q

What enzyme converts citrulline + aspartate –> argininosuccinate? In which cycle?

A
  • arginosuccinate synthase
  • in the urea cycle
36
Q

What vitamin is essential for hydroxyproline and hydroxylysine hydroxylation reactions?

A

vitamin C

37
Q

Name 3 branched-chain aa’s. Which ones are glucogenic or ketogenic?

A
  • leucine (ketogenic)
  • valine (glucogenic)
  • isoleucine (both)
38
Q

What vitamin is essential for aminotransferase function?

A

vitamin B6

39
Q

Which enzyme is vitamin K essential for?

A

gamma-glutanyl carboxylase

40
Q

Why is bilirubin important?

A

it’s an antioxidant

41
Q

Name the ketogenic aa’s.

A
  1. Lysine
  2. Leucine
42
Q

What does carbamoyl phosphatase synthetase I do? In which cycle?

A
  • converts ornithine –> citrulline
  • in the urea cycle
43
Q

What does arginosuccinate lyase do? In which cycle?

A
  • converts argininosuccinate –> arginine
  • in the urea cycle
44
Q

What enzyme catalyzes the conversion of ornithine –> citrulline? What cycle is this part of?

A
  • carbamoyl phosphatase synthetase I
  • the urea cycle
45
Q

Maple Syrup Urine Disease (MSUD) occurs when ______ is deficient, and there is consequently a buildup of the a-keto acids in urine.

A

the branched-chain-a-ketoacid dehydrogenase complex

aDems Unit 2b- Lindsey's (6 decks)

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