1.1.2 Amino Acid Transport & Deamination

Question 1

Gamma-glutamyl cycle enzymes are needed for what?

Answer 1

1. There enzymes constitute and amino acid transport system
2. Involved in the synthesis of glutathione
3. The production of of extracellular gamma glutamyl amino acids which can be taken into cells and converted into 5-oxoproline which stimulates the prevalent form of amino acid transport

Question 2

Describe nitrogen balance and compare positive and negative nitrogen balance.

Answer 2

Question 3

What is the importance of nitrogen balance and ammonia?

Answer 3

Ammonia is toxic to the CNS

Question 4

What can be an outcome if there is a problem in the body’s ability to make urea or eliminate it through the kidneys?

Answer 4

Question 5

What is the importance of benzoic acid and phenylacetate?

Answer 5

These two compounds offer alternative ways of detoxifying and excreting ammonia

Question 6

How is the Urea cycle regulated in the short term and long term?

Answer 6

Question 7

Glutamate dehydrogenase catalyzes the reaction of what?

Answer 7

Glutamate to alpha ketoglutarate

This is important

Question 8

Carbamoyl phosphate combines with ornithine to create?

Answer 8

Citrulline - Mitochondrial processese

Question 9

What are the uses for ammonia?

Answer 9

Question 10

Deficiency in what enzyme leads to Type 2 Hyperammonemia?

Answer 10

Question 11

What is the reaction carried out by carbamoyl phosphate synthetase and why is important about it?

Answer 11

Question 12

What are some of the sources of ammonia?

Answer 12

Intestinal bacterial ureases and ammonia reabsorption

Question 13

What enzyme deficiency will lead to hyperargininemia?

Answer 13

Question 14

Citrulline will eventually be converted into arginine which is a precursor for what?

Answer 14

Urea

Question 15

Deficiency in what enzyme will lead to argininosuccinic aciduria?

Answer 15

Question 16

How are amino groups released from amino acids?

Answer 16

By oxidative reactions or transfer reactions

Question 17

Deficiency in what two enzymes can lead to type one Hyperammonemia?

Answer 17

Question 18

Deficiency in what enzyme can lead to citrullinemia?

Answer 18

Question 19

Glutamate dehydrogenase and amino transferases are involved in?

Answer 19

The synthesis and degradation of amino acids

Question 20

Arginine can be decarboxylated to form what? Why is this important?

Answer 20

It can be decarboxylated to form agmatine - It is an intermediate to forming some polyamines, but also apprears to have neurotransmitter capabilities

Question 21

Glutamine synthetase and glutaminase are involved in?

Answer 21

The storage, production and transport of the equivalent of ammonia