Protein and amino acid metabolism Flashcards

1
Q

Give an example of a glucogenic amino acid.

A

Alanine

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2
Q

Give an example of a ketogenic amino acid.

A

Lysine

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3
Q

Give an example of an amino acid that is both glucogenic and ketogenic.

A

Tryptophan

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4
Q

What effect does insulin have on protein synthesis?

A

It increases protein synthesis

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5
Q

What effect does glucocorticoids such as cortisol have on protein synthesis?

A

It deceases protein synthesis

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6
Q

Why can Cushing’s syndrome lead to striae?

A

There is excess cortisol stimulating excessive protein breakdown

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7
Q

Glutamate is a precursor for …….

A

GABA

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8
Q

Tryptophan is a precursor for serotonin. True or false?

A

True

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9
Q

What are the two main pathways to facilitate nitrogen removal?

A

Transamination and deamination

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10
Q

All aminotransferases are a derivative of vitamin ….

A

Vitamin B6

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11
Q

Which enzyme uses oxaloacetate as a substrate instead of a-ketoglutarate? (Transamination)

A

Aspartate aminotransferase

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12
Q

What is the name of the two key aminotransferases associated with liver function? And what is their role?

A
Alanine aminotransferase (ALT)- Converts alanine to glutamate
Aspartate aminotransferase (AST)- Converts glutamate to aspartate
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13
Q

Where does deamination occur?

A

The liver and the kidney

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14
Q

Which two amino acids can enter the urea cycle?

A

Glutamate and aspartate

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15
Q

Is the urea cycle regulated?

A

No. It is inducible but not regulated.

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16
Q

What is a glucogenic amino acids?

A

Amino acids that can be converted to glucose via gluconeogenesis

17
Q

What are ketogenic amino acids?

A

Amino acids that can be degraded directly into acetyl CoA which is the precursor of ketone bodies.

18
Q

What causes refeeding syndrome?

A

Ammonia toxicity due to the down-regulation of the urea cycle

19
Q

Defects in the urea cycle leads to what?

A

Hyperammonia and accumulation/excretion of urea cycle intermediates

20
Q

What are the symptoms of hyperammonemia?

A

Vomiting, lethargy and irritability

21
Q

Which organ is ammonia very toxic to and why is it toxic?

A

It is toxic to the brain and disrupts cerebral blood flow as well as altering the blood-brain barrier.

22
Q

Ammonia can combine with which amino acid to move from the periphery to the liver or kidney for disposal?

A

Glutamate. It combines with glutamate to form glutamine via Glutamine synthase.

23
Q

Which enzyme is deficient in PKU?

A

Phenylalanine hydroxylase

24
Q

What accumulates in PKU and how is it excreted?

A

Phenylalanine accumulates and phenylketones are consequently seen in the urine

25
What are the symptoms of PKU?
Sever intellectual disability, developmental delay, microcephaly, seizures and hypopigmentation
26
How do you treat PKU?
Low phenylalanine diet
27
What are homocystinurias?
Problems breaking down methionine
28
Which enzyme is commonly defective in homocystinuria?
Cystathionine B-synthase
29
What parts of the body does homocystinuria effect?
Connective tissues, muscles, CNS and CVS
30
How do you treat homocystinurias?
Low methionine diet
31
What would you find in the urine of someone suffering from homocystinuria?
Homocystine
32
What is commonly use an indicator of renal function?
Creatinine