Amino Acid Degradation Flashcards

1
Q

What is the metabolic flow of amino acid nitrogen?

A

Transamination, deamination, and urea formation

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

What is transamination?

A

Transfer of amino group of amino acid to an a-keto acid

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

Transamination requires which enzymes and which co-factors?

A

Enzyme: aminotransferases (transaminases: AST & ALT)
Cofactor: pyroxidal phosphate (Vit B6 derivative)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

What is oxidative deamination?

A

Reversible reaction determined by levels of glutamate, a-ketoglutarate, and ammonia (both catabolic and anabolic reactions.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

What are the enzymes, co-factors, and allosteric regulators of oxidative deamination?

A

Enzyme: glutamate dehydrogenase
Cofactor: NAD or NADP
Allosteric regulators: (+) ADP; (-) GTP

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

What is urea?

A

It is a low toxicity, water-soluable metabolite, enters the blood, and is excreted in urine.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

In which organ does the urea cycle take place?

A

Liver

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

What is the cellular location of the reactions in the urea cycle?

A

First two are in the mitochondrial matrix; the final reactions occur in the cytosol.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

What goes into the reaction?

A

CO2, NH4+, 3 ATP, Aspartate, and H2O

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

What are the products of the urea cycle?

A

Urea, 2ADP, 2 Pi, AMP, PPi, fumerate

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

What is the cellular location of the formation of carbamoyl phosphate from CO2 and ammonia, and what is the energy requirement?

A

Location: mitochondrial matrix

Energy requirement: 2 ATP

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

What are the enzymes and allosteric activators in the formation of carbamoyl phosphate from CO2 and ammonia?

A

Enzyme: carbamoyl phosphate synthetase (CPS-1 - control step)
Allosteric activator: N-acetylglutamate

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

What two products create citrulline?

A

Carbamoyl phosphate and ornithine

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

What are the enzymes and cellular location of the formation of citrulline?

A

Enzyme: ornithine transcarbamoylase

Cellular location of reaction: mitochondrial matrix

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

What is a regulation of the urea cycle?

A

Feeding patterns and need to dispose of nitrogen. Some pattern will still exist during starvation with the breakdown of muscle for fuel.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

What are consequences of disorders in the urea cycle?

A

Hyperammonemia - elevated blood ammonia which is highly toxic to the brain

17
Q

What are treatments of disorders of the urea cycle?

A
  1. ) Low protein diet

2. ) administration of benzoate or phenylacetate

18
Q

Synthesis of argininosuccinate comes from which sources? What is the cellular location of this reaction and what is the energy requirement?

A

It is synthesized from Citrulline and Aspartate in the cytosol.
Energy requirement: 2 high energy phosphate bonds