Amino Acid Metabolism Flashcards

0
Q

What is the primary fuel of the kidneys in normal and fasting conditions?

A

Glutamine

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1
Q

Glutamine is a source of ammonia used to do what?

A

Buffer hydrogen ions

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2
Q

In kidney metabolism, glucose is used mainly by the cells of the ___

A

Renal medulla

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3
Q

In kidney metabolism, lactate is largely oxidized by the cells of the __

A

Renal cortex

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4
Q

What is the major fuel of the gut?

A

Glutamine

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5
Q

Beside glutamine, the gut also oxidizes what two things?

A

Aspartate and BCAA

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6
Q

Are fats and glucose significant energy sources for the gut?

A

Nope

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7
Q

Nitrogens from gut metabolism are transfered where and for what?

A

To the liver for detoxification

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8
Q

Colon cells make extensive use of short-chain fatty acids produced by what?

A

Bacteria

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9
Q

What type of amino acids produce pyruvate or Krebs cycle intermediates?

A

Glucogenic

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10
Q

What type of amino acids produce acetyl CoA or acetoacetyl CoA?

A

Ketogenic

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11
Q

Which amino acids are purely ketogenic? (2)

A

Lucine and Lysine

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12
Q

Which amino acids are both keto- and gluco-genic? (5)

A

Tryptophan, tryosine, phenylalanine, isoleucine, threonine

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13
Q

Which amino acids are branch chain?

A

Valine, leucine, isoleucine

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14
Q

Each BCAA is a substrate for what?

A

A specific aminotransferase

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15
Q

What acts on all three alpha-keto acid products?

A

BCAA dehydrogenase

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16
Q

BCAA dehydrogenase causes what type of reaction?

A

Oxidative decarboxylation

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17
Q

TPP, NADH, FADH2, lipoic acid, and CoA are cofactors for what reaction?

A

Degradation of BCAAs (oxidative decarboxylation)

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18
Q

What results from a defective branched-chain keto acid dehydrogenase?

A

Maple syrup urine disease

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19
Q

What is the precursor of tyrosine?

A

Phenylalanine

20
Q

What is the enzyme that converts phenylalanine to tyrosine?

A

Phenylalanine hydroxylase

21
Q

What is the cofactor in converting phenylalanine to tyrosine?

A

Tetrahydrobiopterin

22
Q

What is the inability to convert phenylalanine to tyrosine?

A

Phenylketonuria

23
Q

What are phenylpyryvate, phenyllactate, and phenylacetate?

A

Toxic phenylketone by-products

24
Q

Which type of PKU is a phenylalanine hydroxylase defect?

A

Classical PKU

25
Q

What is needed to avoid mental retardation with classical PKU?

A

Phenylalanine restricted diet

26
Q

Which type of PKU derives from a defect in tetrahydrobiopterin reductase?

A

Atypical PKU

27
Q

Tyrosine is ultimately catabolized to what two products?

A

Fumarate and acetoacetate

28
Q

What differs following high carbohydrate and high protein meals?

A

Blood glucose and hormone levels

29
Q

What is the ratio for nitrogen balance?

A

Daily losses = daily intake

30
Q

Negative nitrogen balance is when___

A

N excretion is more than consumption

31
Q

Positive nitrogen balance is when ___

A

N excretion is less than consumption

32
Q

Degradation of skeletal muscle protein increases in what condition?

A

Hypercatabolism

33
Q

Gluconeogenesis and synthesis of immune system components increases in what condition?

A

Hypercatabolism

34
Q

Urea synthesis increases in what condition?

A

Hypercatabolism

35
Q

What AA is synthesized by a transamination reaction from pyruvate?

A

Alanine

36
Q

What AA is synthesized from transamination of oxaloacetate?

A

Aspartate

37
Q

What AA is synthesized from amidation of aspartate?

A

Asparagine

38
Q

What AA is synthesized from reductive amination of alpha-ketoglutarate (glutamate dehydrogenase reaction)?

A

Glutamate

39
Q

What AA is synthesized from amidation of glutamate?

A

Glutamine

40
Q

What AA is synthesized from the urea cycle?

A

Arginine

41
Q

What AA is synthesized from glutamate?

A

Proline

42
Q

What AA is synthesized from 3-phosphoglycerate (glycolysis)?

A

Serine

43
Q

What AA is synthesized from serine?

A

Glycine

44
Q

What AA is synthesized from serine and methionine?

A

Cysteine

45
Q

What AA is synthesized from phenylalanine?

A

Tyrosine

46
Q

What cofactors is defective in atypical PKU?

A

Tetrahydrobiopterin

47
Q

Where does TA occur?

A

Cytosol