Chapter 32: Amino Acid Catabolism, Disposal of the Carbon Skeleton Flashcards

1
Q

Consider amino acids in two halves

A
  • Carbon skeleton

- Amino group

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2
Q

Carbon atoms from amino acids enter metabolic pathways

A
  • After the amino group is removed (first step)
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3
Q

AAs that enter glycolysis or the CAC are called

A
  • Glucogenic amino acids
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4
Q

AAs that are converted to FAs or to the keto acids (acetoacetic acid or acetate) are considered

A
  • Ketogenic amino acids
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5
Q

Thirteen amino acids are

A
  • Purely glucogenic
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6
Q

Only AAs that are purely ketogenic

A
  • Leucine

- Lysine

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7
Q

AAs that are both glucogenic and ketogenic can be metabolized to

A
  • Oxaloacetate

- Acetyl-CoA

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8
Q

Surplus AAs are used as

A
  • Metabolic fuel
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9
Q

Alanine, serine, and cysteine are converted to

A
  • Pyruvate
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10
Q

The carbon skeletons of tryptophan, glycine, and threonine can also be converted to

A
  • Pyruvate
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11
Q

Some of the C5 family (glutamine, proline, arginine, and histidine) are converted to

A
  • Glutamate

- Then alpha-ketoglutarate (via oxidative deamination)

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12
Q

Alpha-ketoglutarate is an intermediate of

A
  • The CAC
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13
Q

Aspartate and asparagine (C4) can both be metabolized to

A
  • Oxaloacetate
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14
Q

Asparagine is hydrolyzed to aspartate by

A
  • L-asparaginase
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15
Q

Aspartate can also be converted to

A
  • Fumarate
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16
Q

Three amino acids are converted to fumarate

A
  • Phenyalanine
  • Tyrosine
  • Aspartate
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17
Q

Phenylalanine and tyrosine are degraded by

A
  • Oxygenases

- Degraded to acetoacetate and ….

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18
Q

In mammals, there is no net mechanism for

A
  • Reconverting acetyl-SCoA or acetoacetyl CoA back to glucose
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19
Q

The keto acids generated from these ketogenic amino acids can be

20
Q

Amino acids that are both glucogenic and ketogenic

A
  • Phenylalanine
  • I
  • T
  • T
  • T
21
Q

Phenylalanine and tyrosine are examples of

A
  • Amino acids that are both glucogenic and ketogenic
22
Q

Metabolism of phenylalanine and other aromatic amino acids follows a common sequence of

A
  • Oxidation
  • Deamination
  • Ring opening hydrolysis
23
Q

Branched-chain amino acids

A
  • Isoleucine
  • Leucine
  • Valine
24
Q

All branched-chain amino acids are

A
  • Essential amino acids
25
BC amino acids are partially degraded by
- Serires of irreversible reactions
26
BC amino acid degradation pathway
1. BC amino acid transaminase 2. BC a-keto acid dehydrogenase complex 3. Acyl CoA dehydrogenase
27
BC a-keto acid dehydrogenase complex requires these cofactors
- NAD+ - FAD - Thiamine pyrophopshate - Lipoic acid - Coenzyme A
28
Which branched chain amino acid is ketogenic or glucogenic?
- Leucine is ketogenic
29
PKU is caused by
- An inborn mutation that leads to aberant splicing
30
In PKU mutation of G is replaced by an A, whic occurs in
- Intron 12
31
In PKU mutation of G is replaced by an A, whic occurs in
- Intron 12
32
Phenylalanine hydroxylase activity measures
- Postnatally
33
When phenylalanine hydroxlase fails to mature,
- Brain damage occurs
34
The disease inherited is an
- Autosomal recessive characteristin | - Frequencey of 1:25,000
35
Phenylalanine acts as an
- Antimetabolite......
36
Tyrosinosis
- Detected in one individual | - Useful in determining correct sequence of reactions ....
37
Alcaptonuria
- Lack of homogentisic acid oxidase | - First of all inborn errors of metabolism to be described
38
Alcaptonuria results in
- Accumulation and elimination of homogentisate in the urine
39
When homogentisate is oxidized upon exposure to the air
- It forms a brownish/black pigment
40
Amino acids can be converted into nonprotein substances
- Hormones - Melanin - Neurotransmitters - Nitrogenous bases - Creatine - Nitric oxide - Heme
41
Hormones from AAs
- Tyrosine > catecholamines (dopamine, epi, norepi) & T3 and T4
42
Melanin can be converted to
- Tyrosine
43
Neurotransmitters from AAs
- Tryptophan > serotonin | - Glutamate > GABA and glutathione
44
Nitrogenous bases from AAs
- Purines & pyrimidines from aspartate & glutamine
45
Creatine from AAs
- Arginine & glycine
46
Nitric acid from AA
- Arginine (urea cycle)
47
Heme from AA
- Glycine