Week 14: Special Products from Amino Acids

Cobalamin supplementation would be most appropriate in the case of which of the following enzyme deficiencies?
- Homocysteine methyltransferase
- Cystathionine β-synthase
- Phenylalanine hydroxylase
- Branched-chain keto-acid dehydrogenase
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Cobalamin supplementation would be most appropriate in the case of which of the following enzyme deficiencies?
- Homocysteine methyltransferase
- Cystathionine β-synthase
- Phenylalanine hydroxylase
- Branched-chain keto-acid dehydrogenase
- Homocysteine methyltransferase

Outline the metabolic pathways for tyrosine, phenylalanine, methionine, cysteine and branched-chain amino acids, and identify reactions associated with clinically significant metabolic disorders.
Nitrogen from amino acid metabolism is incorporated into all of the following compounds except:
- Cholesterol
- Nucleotides
- Arginine
- Urea
- Cholesterol

Name the principal catabolites of the carbon skeletons of the protein amino acids and the major metabolic fates of these catabolites
Serotonin is synthesized from which of the following amino acids?
- Threonine
- Valine
- Tryptophan
- Tyrosine
- Tryptophan

Recognize tryptophan metabolites serotonin, melatonin.
A deficiency in vitamin B6 (pyridoxal phosphate) would negatively impact which of the following pathways:
1. Metabolism of cysteine to homocysteine
2. Metabolism of phenylalanine to tyrosine
3. Conversion of methyl malonyl-Co A to succinyl-CoA
- 1 only
- 2 only
- 2 and 3
- 1 and 2
- 1 only

Outline the metabolic pathways for tyrosine, phenylalanine, methionine, cysteine and branched-chain amino acids, and identify reactions associated with clinically significant metabolic disorders.
In an individual with a deficiency in the branched-chain ketoacid dehydrogenase complex, metabolism of which of the following amino acids would not likely be impaired?
- Leucine
- Tyrosine
- Isoleucine
- Valine
- Tyrosine

Identify the specific metabolic defects in hypervalinemia, maple syrup urine disease, intermittent branched-chain ketonuria, isovaleric acidemia, and methylmalonic aciduria
A deficiency in which of the following enzymes would impair the production of S-adenosylmethionine (SAM)?
- Tryptophan synthase
- Methionine adenosyltransferase (MAT)
- Phenylalanine hydroxylase
- Spermidine synthase
- Methionine adenosyltransferase (MAT)

Document the role of S-adenosylmethionine in metabolism.
Hypopigmentation disorder is primarily due to deficient metabolism of which of the following amino acids?
- Methionine
- Cysteine
- Tyrosine
- Arginine
- Homocysteine
- Tyrosine

Outline the metabolic pathways for tyrosine, phenylalanine, methionine, cysteine and branched-chain amino acids, and identify reactions associated with clinically significant metabolic disorders.
Which of the following compounds or substrates is required for the synthesis of tyrosine? (+ indicates the compound is required; - indicates the compound is not required)
1. + Tetrahydrofolate (THF)
+ Tetrahydrobiopterin (BH4)
+ Tryptophan
- Phenylalanine

2. - Tetrahydrofolate (THF)
+ Tetrahydrobiopterin (BH4)
+ Tryptophan
- Phenylalanine

3. + Tetrahydrofolate (THF)
- Tetrahydrobiopterin (BH4)
- Tryptophan
+ Phenylalanine

4. - Tetrahydrofolate (THF)
+ Tetrahydrobiopterin (BH4)
- Tryptophan
+ Phenylalanine
4. - Tetrahydrofolate (THF)
+ Tetrahydrobiopterin (BH4)
- Tryptophan
+ Phenylalanine

Outline the metabolic pathways for tyrosine, phenylalanine, methionine, cysteine and branched-chain amino acids, and identify reactions associated with clinically significant metabolic disorders.