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14 Cards in this Set

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1. Which non-essential AA can be made in a single step?
1. Serine --> GLYCINE

2. Pyruvate --> ALANINE (reversible)

3. Phenylalanine --> Tyrosine

4. α-Ketoglutarate <--> Glutamate
(transamination)

5. Oxalacetate <--> Asparate
(transamination)
2. What are glycine and serine considered?

How do they relate to one another?

What enzymes are necessary

What cofactor is required?
Metabolic partners

Both readily convert

1. Serine hydroxymethyl transferase
2. PLP

FH4
3. What can FH4 do?

How can FH4 be generated?

Why is FH4 necessary?
Can hold individual C atoms in various oxidation states

Cleave glycine w/ glycine cleavage enzyme

So can convert glycine to serine
4. How can the cori cycle be modified to use branched chain AA?
Instead of using lactate to regenerate pyruvate in liver and ultimately glucose, use ALANINE

1. Liver pyruvate
2. Liver glucose
3. Blood glucose
4. Muscle glucose
5. Muscle pyruvate
6. NH2 is added to form alanine
5. What is proline synthesized from?

How does this reaction proceed?
Glutamate

1. Glutamate to glutamate semialdehyde

2. Spontaneous cyclization (P 5C)

3. Form Proline
6. What does the first reaction of glutamate to glutamate semialdehdye require?
Reduction requires ATP and NADPH

**NADPH is also required to go from P-5C to proline
7. Where is glutamate semialdehyde produced?

What does it provide the start for?
(three things)
Gut tissue

1. Proline
2. Ornithine
3. Arginine
8. How is arginine formed from glutamate seminaldehdye (GSA)?
1. Transamination to Ornithine
-ornithine aminotransferase
-aldehyde group to amino group

2. Ornithine to arginine

3. Arginine back to ornithine (produce urea)
9. What is the overall metabolism of branched chain AA?

Where does this occur?

How are the pathways for each branched chain AA?
1. Aminotransferase
-each AA has special transferase

2. Decarboxylating dehydrogenase

3. Stuff happens

4. Final fates

In muscle tissue during fasting

Pathways are different for each AA
10. Describe valine metabolism?
1. Transamination of valine

2. Oxidative decarboxylation

3. Propionyl CoA (product of odd chain FA oxdiation)

4. Gluconeognenic
11. Describe the metabolism of isoleucine.
1. Transamination of isoleucine

2. Acetyl CoA --> ketogenic

3. Propionyl CoA ---> gluconeogenic
12. Describe the metabolism of leucine?
1. Transmaination of leucine

2. Acetoacetate ---> ketogenic

3. Acetyl CoA ---> ketogenic
13. What is maple syrup urine disease?

When does it present?
Typical inherited disorders of AA metabolism

**low incidence worldwide but abundant among subpopulations (i.e. SE PA)

After first protein meal (under maternal circulation in womb so everything's fine)
14. What are symptoms of maple syrup urine disease?

What is the solution to this?
1. Failure to thrive
2. Feeding difficulties
3. Mental development affected
4. Convulsions

Cut back branched chain AA but can't cut out altogether b/c need essential AA (engineer diet)