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Biochemistry > Endo: Catabolism Of carbon skeleton Of AA > Flashcards

Endo: Catabolism Of carbon skeleton Of AA Flashcards

0
Q

AAs that don’t undergo transamination

A

Proline, Hydroxyproline, Lysine & Threonine “PaHaLaTa”

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1
Q

First catabolic reaction of AA catabolism

A

Removal of a-amino nitrogen by transamination

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2
Q

AA are converted into _________ or their precursor so that they can be metabolized to ___ & ___ or used in ______. Accounts for ____ of metabolic energy generated by animals.

A

CAC intermediates. CO2 & H2O. Gluconeogenesis. 10-15%.

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3
Q

Carbon skeletons are degraded into Pyruvate, succinyl coa, oxaloacetate or fumarate and a-ketoglurate and are therefore glucose precursor. “Pa SOFA”

A

Glucogenic AA

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4
Q

Carbon skeletons are broken down to acetyl coA or acetoacetate and can thus be converted to fatty acids or ketone bodies.

A

Ketogenic AA

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5
Q

Only ketogenic AA

A

Leucine

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6
Q

Glucogenic and Ketogenic AA at the same time

A

Phenylalanine, Lysine, Isoleucine, Tryptophan & Tyrosine “PLITT”

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7
Q

AA converted to pyruvate

A

Cysteine, Threonine, Tryptophan, Alanine, Glycine & Serine “CT TAGS”

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8
Q

AA coverted to oxaloacetate

A

Aspartate & Asparagine “AA”

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9
Q

AA converted to a-ketoglutarate

A

Proline, Histidine, Arginine, Glutamate & Glutamine “Pa HAGG”

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10
Q

AA converted to fumarate

A

Phenylalanine, Aspartate & Tryptophan “PAT”

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11
Q

AA converted to succinyl coA

A

Valine, Isoleucine & Methionine “VIM”

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12
Q

AA degraded to acetyl coA or acetoacetate

A

Phenylalanine, Isoleucine, Leucine, Lysine, Tryptophan, Threonine & Tyrosine “PILL T3”

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13
Q

Transamination forms pyruvate which can then be decarboxylated to acetyl CoA

A

Alanine

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14
Q

Splits to CO2 and NH4 and N5, N10 Methylene Tetrahydrofolate

A

Glycine

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15
Q

Transamination of glycine to ______ with Glu or Ala

A

Glyoxylate

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16
Q

Failure to catabolize glyoxylate leads to

A

Hyperoxaluria

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17
Q

Degraded to glycine and N5, N10 methylene tetrahydrofolate

A

Serine

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18
Q

Enzyme used to convert serine to pyruvate

A

Serine hydratase

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19
Q

Converted to cysteine

A

Cystine

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20
Q

Cysteine is catabolized via what pathways

A

Direct oxidative pathway & Transamination pathway

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21
Q

Enzyme used to convert cystine to cysteine

A

Cystine reductase

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22
Q

Cysteine: direct oxidative pathway

A

Cysteine [cysteine deoxygenase] cysteine sulfinate [transaminase] sulfinyl pyruvate [desulfinase] pyruvate

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23
Q

Cysteine: transamination pathway

A

Cysteine [transaminase] 3 mercaptopyruvate = pyruvate & 3 mercaptolactate

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24
Q

Cleaved to acetaldehyde and glycine

A

Threonine

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25
Q

Oxidized to acetate which is then converted to acetyl CoA

A

Acetaldehyde

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26
Q

Pathway of Threonine

A

Threonine [threonine aldolase] acetaldehyde [aldehyde dehydrogenase] acetate [acetate thiokinase] acetyl CoA

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27
Q

Transamination forms oxaloacetate

A

Aspartate

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28
Q

Undergoes hydrolysis to aspartate

A

Asparagine

29
Q

Aspartate and Asparagine pathway

A

Asparagine [asparaginase] Aspartate [transaminase] Oxaloacetate

30
Q

Transamination forms a-ketoglutarate

A

Glutamate

31
Q

Undergoes hydrolysis to glutamate

A

Glutamine

32
Q

Glutamate and Glutamine Pathway

A

Glutamine [glutaminase] Glutamate [transaminase] a-Ketoglutarate

33
Q

Oxidized to dehydroproline which then adds water forming ___________. This is then oxidized to _______ and transaminated to _________.

A

Proline. Glutamate y-semialdehyde. Glutamate. a-ketoglutarate.

34
Q

Proline pathway

A

Proline [proline dehydrogenase] glutamate-y semialdehyde [glutamate semialdehyde dehydrogenase] Glutamate = a-ketoglutarate

35
Q

Converted to ornithine which then undergo transamination to glutamate-y semialdehyde

A

Arginine

36
Q

Arginine pathway

A

Arginine [arginase] ornithine

37
Q

Non-oxidatively deaminated then hydrated and its imidazole ring cleaved to form ___________; formimino group is then transferred to TH4 to form ______ and ______.

A

Histidine. N-formiminoglutamate(figlu). N-formiminoTH4 & Glutamate.

38
Q

Histidine pathway

A

Histidine [histidinase] Urocanate [urocanase] 4-imidazolone-B-proprionate [imidazolone proprionate hydrolase] N-formiminoglutamate [glutamate formimino transferase] Glutamate = a-Ketoglutarate

39
Q

Can be used to detect folic acid deficiency

A

Excretion of FIGLU ff. a dose of histidine

40
Q

First reaction in its degradation is its hydroxylation to tyrosine

A

Phenylalanine

41
Q

Transaminated to p-hydroxyphenyl-pyruvate followed by concerted ring hydroxylation and side chain migration to form ______ with _____ as reductant; aromatic ring opens and is hydrolyzed to ______ and _______.

A

Homogentisate. Ascorbate. Fumarate & Acetoacetate.

42
Q

An important methyl donor

A

S-Adenosyl-methionine (SAM)

43
Q

Condenses with ATP to form SAM

A

Methionine

44
Q

Methionine to SAM pathway

A

Methionine+ATP [methionine adenosyl transferase] SAM

45
Q

In methionine, removal of methyl group forms ________ which is hydrolyzed to _______ & _______.

A

S-adenosylhomocysteine. Adenosine & Homocysteine.

46
Q

In methionine, homocysteine combines to serine to yield _______ which is subsequently forms ______ and _______.

A

Cystathione. Cysteine & a-ketobutyrate.

47
Q

In methionine, a-Ketobutyrate is degraded to _______ & then to ______.

A

Propionyl CoA. Succinyl CoA.

48
Q

Methionine pathway

A

Methionine- S adenosyl methionine- S adenosyl homocysteine- Homocysteine plus serine [cystathione synthase] Cystathione = cysteine and a-Ketobutyrate= proprionyl CoA

49
Q

Isoleucine is converted to

A

Propionyl CoA

50
Q

Valine is converted to

A

Methylmalonyl CoA

51
Q

Methionine is converted to

A

a-Ketobutyrate

52
Q

a-Ketobutyrate to Propionyl CoA to Methylmalonyl CoA to

A

Succinyl CoA

53
Q

Has several pathways for degradation but the pathway that proceed via the formation ______ predominates in mammalian liver.

A

Lysine. Saccharopine.

54
Q

Pathway of lysine involves

A

Transamination, oxidative decarboxylation and reactions similar to fatty acyl CoA oxidation. “TOF”

55
Q

Lysine pathway

A

Lysine- saccharopine- aminoadipate semialdehyde- aminoadipate- ketoadipate- glutaryl CoA- crotonyl CoA- B hydroxybutyryl coA- Acetoacetyl CoA- HMG CoA- Acetoacetate

56
Q

Initial reaction involves cleavage of indole ring with incorporation of 2 atoms of molecular oxygen by ________.

A

Tryptophan. Tryptophan oxygenase.

57
Q

In tryptophan, carbon atoms of side chain and aromatic ring completely degraded via

A

Kynurenine-anthranilate pathway

58
Q

An iron porphyrin metalloprotein. Inducible in the liver by adrenal corticosteroid and by tryptophan.

A

Tryptophan oxygenase

59
Q

Tryptophan oxygenase feedback is inhibited by

A

Nicotinic acid derivatives including NADPH

60
Q

Purely ketogenic AA. Degraded to _____ which is converted to _____ and ______.

A

Leucine. HMG CoA. Acetoacetate & Acetyl CoA.

61
Q

Brached chain AA

A

Leucine, Isoleucine and Valine “LIV”

62
Q

Shares the same first three reaction that employs a common enzyme. Resulting products are then catabolized by distinct pathways.

A

Branched chain AA

63
Q

Same first 3 reactions shared by Branched chain AA

A

-Transamination to corresponding a-Ketoacids
-Oxidative decarboxylation to corresponding acyl CoA
-Dehydrogenation by FAD to form a double bond
“TOD”

64
Q

Almost always associated with AA catabolism rather than AA biosynthesis. Sufficient amount of AA whether essential or non essential are present in a well balanced diet.

A

Amino acidopathies

65
Q

Failure to catabolize AAs will result in accumulation of AA and its metabolites to the point that they become _____.

A

Toxic

66
Q

Defect in the catabolism of ______. Deficiency in the enzyme ________. Most striking manifestation is darkening of urine d/t the presence of homogentisate. Later develops arthritis and connective tissue pigmentation.

A

Alkaptonuria. Tyrosine. Homogentisate oxidase.

67
Q

Results in the inability to covert phenylalanine to tyrosine. Defect may be in the enzyme ________(classic PKU), ______ or ______. Major consequence is mental retardation.

A

PKU. Phenylalanine hydroxylase, Tetrahydrobiopterine synthase or Dihydrobiopterine synthase.

68
Q

Treatment for PKU

A

Diet low in phenylalanine

69
Q

Alternative pathways of phenylalanine catabolism

A

Phenylketonurics

70
Q

Defect in the intestinal and renal transport of neutral AA including tryptophan. Manifest with pellagra-like s/sx because of limited conversion of tryptophan to niacin.

A

Hartnup disease

71
Q

Defect in the absence of branched chain a-ketoacid Dehydrogenase. Odor of urine resembles maple syrup or burnt sugar. Brain damage develops unless promptly treated with ________.

A

Maple syrup urine disease. Diet low in BCAA.

Biochemistry (20 decks)

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