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Biochem > Amino Acid Degredation > Flashcards

Amino Acid Degredation Flashcards

1
Q

Ketogenic

A
  • Amino acids that are degraded to acetoacetyl-CoA . acetyl-CoA
  • Will eventually form ketone bodies
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2
Q

Glucogenic

A
  • Amino acids that are degraded to pyruvate, α-ketoglutarate, succinyl-CoA, fumarate, and oxaloacetate
  • Will eventually enter citric acid cycle
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3
Q

How many amino acids are ketogenic?

A

7

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4
Q

How many amino acids are glucogenic?

A

18

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5
Q

What are the ketogenic amino acids?

A
  • Leucine
  • Lysine
  • Tyrosine
  • Isoleucine
  • Phenylalanine
  • Threonine
  • Tryptophan
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6
Q

What are the glucogenic amino acids?

A
  • Alanine
  • Arginine
  • Asparagine
  • Aspartate
  • Cysteine
  • Glutamine
  • Glycine
  • Proline
  • Serine
  • Histidine
  • Methionine
  • Valine
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7
Q

What are the 5 amino acids that are both glucogenic and ketogenic?

A
  • Tyrosine
  • Isoleucine
  • Phenylalanine
  • Threonine
  • Tyrptophan
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8
Q

What are the 2 exclusively ketogenic amino acids?

A
  • Leucine
  • Lysine
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9
Q

What enzymes play an important role in amino acid catabolism?

A
  • Biotin
  • THF
  • S-adenosylmethionine (adoMet)
    All specialize in one carbon transfer reaction
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10
Q

THF (Tetrahydrofolate)

A
  • Two nitrogen atoms are important
  • Carbon group undergoing transfer can be bonded to N5, N10 or both
  • Three different forms
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11
Q

What are the three different forms of THF

A
  1. N5, N10-Methylenete-THF
  2. N5-Methy-THF
  3. N5, N10-Methenyl-THF
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12
Q

N5, N10-Methylenete-THF

A
  • Primary source of one carbon units comes from the breakdown of serine to glycine
  • Transfers medium oxidized carbons (CH2OH)
  • Is interconverted between N5-Methy-THF and N5, N10-Methenyl-THF
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13
Q

N5-Methy-THF

A

Transfers the most reduced carbon (CH3)

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14
Q

N5, N10-Methenyl-THF

A

Transfers a more oxidized carbon (CHO)

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15
Q

What is N10-Formyl-THF

A

Occurs when carbon atom comes from formate instead of serine

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16
Q

S-adenosylmethionine (adoMet)

A
  • Preferred cofactor for transfer reactions involving methyl groups
  • Synthesized by adenosine and methionine by THF
17
Q

Describe the synthesis of adoMet

A
  1. Methionine + ATP get linked together to form SAM (S-adenosylmethionine)
  2. Methyl group from SAM gets transferred to an acceptor to form S-adenosylhomocysteine
  3. S-adenosylhomocysteine is broken down to homocysteine and adenosine
  4. Methyl group is transferred to homocysteine by N5-Methyl-THF in a reaction that is catalyzed by methionine synthase (this regenerates methionine)
  5. Methionine gets reconverted back into SAM
18
Q

Biotin

A

Transfers carbon atoms from bicarbonate (CO2) in blood in a two step reaction

19
Q

Describe the two step process that is necessary for the one carbon transfer reaction involving Biotin

A

Biotin is linked to the enzyme biotin carboxylase
- Step 1: Biotin reacts with bicarbonate + ATP to replace H on Nitrogen with a CO2 moiety
- Step 2: The CO2 moiety is transferred to another molecule
(ex: acetyl-CoA) producing a carboxylate group on that molecule
(ex: acetyl-CoA —> malonyl-CoA)

20
Q

What amino acids are degraded to pyruvate?

A
  • Alanine
  • Glycine
  • Cysteine
  • Serine
  • Threonine
  • Tryptophan
21
Q

What amino acids are degraded to α-ketoglutarate?

A
  • Arginine
  • Glutamate
  • Glutamine
  • Histidine
  • Proline
22
Q

What amino acids are degraded to succinyl-CoA?

A
  • Isoleucine
  • Methionine
  • Threonine
  • Valine
23
Q

What amino acids are degraded to fumarate?

A
  • Phenylalanine
  • Tyrosine
24
Q

What amino acids are degraded to oxaloacetate?

A
  • Aspartate
  • Asparagine
25
Q

What are the branched chain amino acids?

A
  • Leucine
  • Isoleucine
  • Valine
26
Q

Describe the degradation of branched chain amino acids

A
  • 2 Steps:
  • Step 1:
    Amino acid form —> Keto acid form (PLP aminotransferase reaction)
  • Step 2:
    Keto acid form —> Final product (any of the three) by BCKDH
    This is an oxidative decarboxylation reaction
27
Q

Maple syrup urine disease

A
  • Defective BCKDH enzyme
  • You cannot successfully metabolize branched chain amino acids which results in the accumulation of these amino acids and their “spill over” into the urine
  • Result is thick sweet smelling urine
28
Q

Describe the genetic defect that occurs as a result of the inability to break down phenylalanine

A
  • Phenylalanine hydroxylase = first enzyme in the catabolic pathway for phenylalanine (breakdown of phenylalanine to tyrosine)
  • A defect in the enzyme = PKU disease
  • Result = elevated levels of phenylalanine in the blood
29
Q

What happens in individuals who have PKU which results in elevated levels of phenylalanine in the blood?

A
  • A normally little-used pathway is activated in which phenylalanine undergoes transamination with pyruvate to yield phenylpyruvate
  • Phenylalanine and phenylpyruvate accumulate in blood and tissues impairing the normal development of the brain

Biochem (22 decks)

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