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MCBM > Amino Acid Oxidation > Flashcards

Amino Acid Oxidation Flashcards

1
Q

What can the 7 intermediates produced from the aa be subdivided into

A
  1. Glucogenic: TCA cycle intermediates that can be shunted to gluconeogenesis
  2. Ketogenic: AcetylCoA derivates that can be used for ketone body synthesis.
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2
Q

18/20 aa are at least partially______. 4/20 glucogenic and ketogenic products include ____2/20 are purely ketogenic.

A

glucogenic. 3 aromatics + Ile; Leu and Lys

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3
Q

Methionine breaks down into ____. Phenylalanine breaks down into ______

A

cysteine; tyrosine

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4
Q

The key to interconversion of aa & carbs lies in the interrelationship between aa and:

A

alpha keto acids

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5
Q

What are the 7 metabolites of aa metabolism in glucogenic and ketogenic

A

Glucogenic:

  1. pyruvate
  2. oxaloacetate
  3. alpha KG
  4. fumerate
  5. propionylCoA

Ketogenic:

  1. acetoacetyl CoA
  2. acetylCoA
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6
Q

Which aa form into pyruvate

A
  1. Alanine
  2. Serine
  3. Cysteine
  4. Tryptophan
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7
Q

3C aa form a 3 C alpha keto acid to make

A

pyruvate

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8
Q

What are the 4C aa that are metabolized to make oxaloaceate

A
  1. Asparagine

2. Aspartate

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9
Q

What are the 5C aa that are metabolized to make alpha keto Glutarate

A
  1. Glutamine
  2. Proline
  3. Argenine
  4. Histidine
  5. Glutamate
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10
Q

Which branched chain aa make up succyinyl CoA and acetyl CoA

A
  1. Ile
  2. Valine
  3. Leucine
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11
Q

Valine gets converted to propionyl CoA which means its strictly

A

glucogenic

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12
Q

Ile gets converted to propionylCoA + acetylCoA which means its both:

A

glucogenic and ketogenic

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13
Q

Leu gets converted to acetoacetate which means its strictly

A

ketogenic

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14
Q

Branched chain aa share the first steps of:

A

oxidation. Get transaminated via branched chain aminotransferase

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15
Q

Which aa make up succinylCoA

A
  1. Methionine

2. Threonine

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16
Q

Breakdown of methionine produces:

A

cysteine

17
Q

Met & Thr yield alpha keto butyrate that is converted to

A

propionylCoA.

18
Q

PropionylCoA gets converted to:

A

succinylCoA

19
Q

What does methlmalonyl CoA mutase need

A

cobalmin (B12)

20
Q

What is munechausen syndrome by proxy

A

making people get purposefully sick to get attention

21
Q

What are the two aa that are stricly ketogenic

A

Lycine and leucine

22
Q

Which aa make acetylCoA

A

leucine and lysine

23
Q

what aa make fumarate and acetylCoA

A

aromatic aa:

  1. Phe
  2. Tyr
  3. Trp
24
Q

aromatic aa are both glucogenic and

A

ketogenic

25
Q

What enzyme is required in Phe metabolism. What can result when you have deficiency of this enzyme

A

phenylalanine hydroxylase. PKU

26
Q

What is the enzyme needed in homogentisate

A

homogentisate 1, 2 dioxygenase. Alkaptonuria can result if deficient.

27
Q

what is PKU

A
  1. autosomal recessive disease
  2. deficiency in phenylalaline hydroxylase
  3. all newborns tested at birth
  4. treatment req low phenylalaline diet until adolescene
  5. lack of treatment results in severe mental retardation.
28
Q

What is a cofactor in many hydroxylase reactions.

A

Tetrahydrobiopterin. IMportant for neurotransmitters.

29
Q

what does alkaptonuria cause

A

pigment buildup throughout the body and arthiritis like symptoms.

30
Q

a deficiency in what enzyme results in albinism

A

tyrosinase

31
Q

In humans what is glycine converted to

A

CO2 and NH4

32
Q

In liver, what can serve as a substrate for lactate dehydrogenase and gets converted to oxalate

A

glyoxylate

33
Q

what is the major component of kidney stones

A

Calcium oxalate

34
Q

which aa are both glucogenic and ketogenic

A

3 aromatics: Phe, Tyr, Trp

1 branched chain: Ile

35
Q

Which 2 aa are strictly ketogenic

A

leucine and lysine

36
Q

What are inborn errors of metabolism

A
  1. PKU: defect in phylalanine hydroxylase
  2. Alkaptonuria: defect in homogentisate oxidase
  3. maple syrup urine disease: defect in branched chain amino acid DH
  4. methylmalonic acidemia: defect in methylmalonylCoA mutase
  5. Homocystinuria: defect in cystathione synthase
37
Q

How do you screen for inborn errors of metabolism

A
  1. carrier status: preconception testing of parents
  2. pre-implantation: test IVF embryos prior to implantation
  3. Pre natal: Chorionic villus (10-12 week of pregnancy), amniocentesis (15-18 week of pregnancy) cordocentesis (after 17th week of pregnancy)

MCBM (59 decks)

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