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Biochemistry Block 3 > Catabolism Of Carbon Skeleton > Flashcards

Catabolism Of Carbon Skeleton Flashcards

1
Q

Which two amino acids give you ketone bodies?

A

Leucine and lysine

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2
Q

Conversion of the remaining amino acid carbon skeleton can give you what 3 things?

A

Energy (TCA cycle intermediates), glucose in liver, or ketone bodies

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3
Q

What TCA intermediates can catabolism of carbon skeleton give you (4)

A

Alpha ketoglutarate, succinyl CoA, fumarate, or oxaloacetate

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4
Q

Arginine

  1. Essential or nonessential
  2. Cleaved to produce
  3. Eventually converted to?

~not that important

A
  1. Nonessential
  2. Ornithine
  3. Alpha ketoglutarate
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5
Q

FIGLU reacts with?

FIGLU is in which amino acid reaction?

FIGLU test tells you what?

A

Folic acid (needed for nucleotide synthesis)

Degradation of Histidine into alpha ketoglutarate

If there is a folic acid deficiency (leads to neural tube defects-spina bifida)

~so you can use histidine to detect folic acid deficiency

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6
Q

What amino acid forms pyruvate?

A

Alanine (transamination) - also cystine can

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7
Q

What amino acid forms succinyl CoA

A

Methionine

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8
Q

What should you think of when you hear 1 carbon metabolism?

A

SAM: S-adenosyl methionine

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9
Q

What does SAM do?

Two examples of what SAM products are

What does SAM require

A

Transfers its methyl group to acceptor molecules (makes methylated products)

Epinephrine, melatonin, and creatine

Vitamin B12

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10
Q

5 aminoacidopathies?

A

Homocystinuria, maple syrup urine disease, hyperphenylalaninemias, alkaptonuria, albinism

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11
Q

Homocysteine metabolism

  1. Requires what 2 things?
  2. Why is homocysteine important?
  3. How do you lower homocysteine levels?
A
  1. Vitamin B12 and PLP
  2. Increased levels can lead to MI or stroke in someone in their 20s (atherosclerosis)
  3. By eating greens
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12
Q

Things increased homocysteine cause:

  1. Nitric oxide dysfunction which causes
  2. Irritated blood vessels which causes
  3. Endothelial injury which causes
  4. Increased oxidative damage which causes
A
  1. Vasoconstriction
  2. Inflammation
  3. Clot formation
  4. Atherosclerosis
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13
Q

Besides MI/stroke, what else can homocysteine cause in young people

A

Alzheimer’s and rheumatoid arthritis and neural tube defects

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14
Q

Homocystinuria

  1. What enzyme is deficient
  2. High plasma/urinary levels of __ and low levels of __
  3. Symptoms
  4. Treatment
A
  1. Cystathionine synthetase
  2. High levels of homocysteine and methionine and low levels of cysteine
  3. Mental retardation, osteoporosis, ectopia lentis (dislocation of lens)
  4. Diet low in methionine and supplementation of B6, B12, and folate
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15
Q

Maple syrup urine disease:

  1. Reduced activity of what enzyme
  2. Elevated plasma and urinary levels of what three amino acids
  3. Symptoms are seen?
  4. Symptoms?
A
  1. Branched chain alpha keto acid dehydrogenase
  2. Leucine, isoleucine, and valine (and their alpha keto acids)
  3. 2-3 weeks after birth
  4. Sugary urine smell, infant difficult to feed, extensive brain damage occurs in surviving children (usually die after a year tho)
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16
Q

Phenylalanine metabolism:

  1. Pheylalanine gets converted to which amino acid?
  2. By which enzyme?
A
  1. Tyrosine

2. Phenylalanine hydroxylase

17
Q

Which of the following is an essential amino acid: phenylalanine or tyrosine?

A

Phenylalanine

Tyrosine is nonessential only in the presence of the adequate dietary phenylalanine

18
Q

Deficiency of phenylalanine hydroxylase will cause what disorder?

A

PKU (phenylketonuria) / Type 1 hyperphenylalaninemia

19
Q

Other types of hyperphenylalaninemia can be caused by a deficiency in which enzymes?

A

Biopterin (BH2) reductase / biopterin (BH2) synthase

20
Q

PKU

  1. High levels of what in tissues/plasma/urine
  2. Low levels of?
  3. Feature of urine? Why?
A
  1. Phenylalanine
  2. Tyrosine
  3. Mousy/musty odor due to elevated levels of phenylacetate, phenylactate, and phenylpyruvate (path that creates these 3 normally does not happen, but it will due to abnormal accumulation of phenylalanine)
21
Q

Symptoms of PKU

What test diagnoses PKU? When is this test done?

A

Mental retardation, seizures, psychoses, hypopigmentation

Guthrie test - done after 2-3 days of milk feeding

22
Q

Why would PKU lead to hypopigmentation?

A

Because if you have PKU, phenylalanine is not being converted to tyrosine and tyrosine is the precursor for melanin

23
Q

Treatment for PKU

A

Decrease amount of phenylalanine in diet (not absence since its essential); tyrosine must be supplied in the diet

24
Q

Tyrosinemia type I

  1. Accumulation of?
  2. Deficiency in what enzyme?
  3. Urine symptom?
  4. Treatment?
A
  1. Fumaryl acetoacetate and its metabolites
  2. Fumaryl acetoacetate hydrolase (last enzyme in tyrosine metabolism)
  3. Cabbage like odor
  4. Dietary restriction of phenylalanine and tyrosine
25
Q

Alkaptonuria

  1. Enzyme deficiency?
  2. At what age does it show symptoms
  3. Urine symptom?
  4. What can it cause
A
  1. Homogentisate oxidase
  2. 40
  3. Colored (darkened)
  4. Large joint arthritis
26
Q

What is onchronosis?

What causes it?

A

Pigmentation of cartilage and connective tissues

Oxidation of homogentisate (Alkapton bodies)

27
Q

Tyrosine is precursor for

A

Neurotransmitters (dopamine, epinephrine, NE)

28
Q

What does dopamine help do

Deficient dopamine will cause

A

Chemical messenger that control muscle movement

Parkinsons

29
Q

Albinism is caused by deficient ?

Symptoms

A

Tyrosinase (so decreased production of melanin)

Complete absence of pigment, vision defects, photophobia

30
Q

Tyrosine also gives rise to what hormone?

A

Thyroid hormone

Biochemistry Block 3 (11 decks)

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