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Biochemistry FA > Amino Acids > Flashcards

Amino Acids Flashcards

0
Q

Form of amino acids that are found in proteins

A

L form

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1
Q

Essential amino acids groups

A
  1. Glucogenic
  2. Glucogenic/ketogenic
  3. Ketogenic
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2
Q

All…..amino acids need to be supplied in the diet

A

Essential

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3
Q

A ketogenic amino acid is an amino acid that….

A

can be degraded directly into acetyl CoA through ketogenesis

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4
Q

Essential glucogenic amino acids

A
  1. Methionine (Met)
  2. Valine (Val)
  3. Histidine (His)
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5
Q

Glucogenic amino acid is an amino acid that…..

A

Can be converted into glucose through gluconeogenesis

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6
Q

Essential ketogenic amino acids

A
  1. Leucine (Leu)

2. Lysine (Lys)

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7
Q

Essential glucogenic/ketogenic amino acids

A
  1. Isoleucine (ile)
  2. Phenylalanine
  3. Threonine
  4. Tryptophan
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9
Q

Acidic amino acids and charge

A 
Aspartic acid (Asp)
Glutamic acid (Glu)
both negative
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10
Q

Basic amino acids and charge

A

Arginine (Arg) - positive
Lysine (lys) - positive
Histidine (his) - no charge

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10
Q

Amino acids required during periods of growth

A

Arginine

Histidine

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11
Q

The most basic amino acid

A

Arginine

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12
Q

Amino acids of histones

A

Arginine

Lysine

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13
Q

Amino acid catabolism result in the formation of

A

Common metabolites (pyruvate, acetyl CoA e.g.), which serves as metabolic fluids

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14
Q

Amino acid catabolism result in excess

A

Nitrogen (NH3)

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15
Q

Nitrogen is converted to

A

Urea and excreted by the kidneys

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16
Q

Urea cycle - reactions and locations

A
  • CO2 + NH3 + 2ATP –> carbamoyl phosphate + 2ADP + P ( carbamoyl phosphate synthase 1 and N-acetygkutamate as a cofactor) (Mitochondria)
  • Carbamoyl phosphate + ornithine –> citrulline Ornithine transcarbamylase) (Mitochondria)
  • Citruline + aspartate + ATP–> AMP + PPi + Argininosuccinate (arginossuccinate synthetase)
    Cytoplasm
  • Argininosuccinate –> arginine and fumarate (arginosuccinase) Cytoplasm
  • arginine + H20 –> UREA + Ornithine (Arginase) cytoplasm
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17
Q

Urea productuon reaction

Location

A

Arginine + H2O –> ornithine + Urea
Enzyme arginase
Location:cytoplasm of liver

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18
Q

Urea production location

A

Cytoplasm of liver

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19
Q

Location of ornithine transcarbamylase action

A

Mitochondria of the liver

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20
Q

Ornithine transcarbamylase reaction

Location

A

Carbamoyl phosphate + ornithine –> citrulline

Mitochondria

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21
Q

Arginosuccinate: next step

A

To arginine and fumarate (arginosuccinase)

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22
Q

Urea structure

A 
NH2  (NH3)
         |
        C=O  (CO2)
         |
        NH2  (aspartate)
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23
Q

Arginosuccinate: next step

A

To arginine and fumarate (arginosuccinase)

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24
Q

Urea is excreted by

A

Kidney

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25
Q

Urea cycle site

A

Both Mitochondria and cytoplasm of the liver

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26
Q

Enzyme responsible for urea production

A

Arginase

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27
Q

Aminoacids(NH3) to a-ketoacids causes

A

A ketoglutarate to glutamate(NH3)

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28
Q

Glutamate(NH3) to a-ketoglutorate causes

A

Pyruvate to alanine (NH3)

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29
Q

Cori cycle

A

Glucose to pyruvate to lactate (muscle)
Lactate to pyruvate to glucose (liver)
Glucose back to muscle

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30
Q

Alanine cycle (cahill cycle)

A

Pyruvate-alanine (muscle)
Alanine(NH3) to pyruvate (liver) converting a-ketoglutorate to glutamate(NH3)
pyruvate to glucose (liver)
Liver back to muscle

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31
Q

Hyperammonemia can be divided to

A
  1. Acquired (liver disease)

2. Hereditary (urea cycle enzyme deficiencies

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32
Q

Transport of ammonia by

A

Alanine and glutamate

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33
Q

Hyperammonemia results in

A

Excess NH4+

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34
Q

Excess NH4+ in hyperammonemia results in

A

a-ketoglutarate depletion, leading to inhibition of TCA cycle

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35
Q

Carbamoyl phosphate synthetase I cofactor

A

N-acteylglutammate

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36
Q

Hyperammonemia treatment

A
  1. Limit protein diet
  2. Phenylbutyrate or Benzoate (bind aminoacid and lead to excretion) may be given to decrease ammonia levels
  3. Lactulose to acidify the GI tract and trap NH4+ for exertion
  4. Rifamixin to decrease colonic ammoniagenic bacteria
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37
Q

N-acetyglutamate deficiency leads to

A

Hyperammonemia

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38
Q

N-acetyglutamate deficiency is identical to

A

Carbamoyl synthetase I deficiency

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39
Q

Ornithine trancarbamylase deficiency mode of inheritance

A

XR (vs other urea cycle enzyme deficiency which are autosomal recessive)

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40
Q

Lab findings that suggest N-acetyglutamate deficiency and clinical presentation

A

Increased ornithine with normal urea cycle enzymes

  1. poorly regulated respiration and body Q
  2. poor feeding
  3. developmental delay
  4. intellectual disability
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41
Q

Most common urea cycle disorder

A

Ornithine trancarbamylase deficiency

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42
Q

Urea cycle enzymes deficiencies are AR except

A

Ornithine trancarbamylase deficiency –> XR

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43
Q

Ornithine trancarbamylase deficiency / pathophysiology

A

Interferes with the body’s ability to eliminate ammonia

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44
Q

Ornithine trancarbamylase deficiency/ often evident in (age)

A

First few days of live, but may present with late onset

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45
Q

Ornithine trancarbamylase deficiency /what is happening with the excess carbamoyl phosphate

A

Is converted to orotic acid

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46
Q

Ornithine trancarbamylase deficiency findings

A
  1. Increased orotic acid blood and urine
  2. Decreased BUN
  3. Symptoms of hyperammonemia
  4. NO MEGALOBLASTIC ANEMIA (vs orotic aciduria)
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47
Q

Orotic aciduria vs Ornithine trancarbamylase deficiency (lab)

A

Ornithine trancarbamylase deficiency has no megaloblastic anemia

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48
Q

Amonoacid and vitamin to heme

A

Glycine to porphyrin (B6)

Porphyrin to heme

49
Q

Amina acid responsible for heme synthesis

Vitamin responsible for heme synthesis

A

Glycine

B6

50
Q

Histamini synthesis (amino acid and vitamin)

A

Histidine to histamine (B6)

51
Q

Tryptophan participate in the synthesis of

A

Niacin (NAD, NADP)
Serotonin
Melatonin

52
Q

Ammonia intoxication

A
  1. Slurring of speech
  2. Tremor (asterixis)
  3. Somnolence
  4. Vomiting
  5. Cerebral edema
  6. Blurring vision
53
Q

Arginine is responsible for the synthesis of

A
  1. Creatinine
  2. Urea
  3. Nitric oxide (BH4 as a cofactor)
54
Q

Synthesis of melatonin (aminoacid and vitamin)

A

Tryptophan to serotonin (B6+BH4)

Serotonin to melatonin

55
Q

Nitric oxide synthesis (amino acid and coafactor)

A

Arginine to nitric oxide (BH4)

56
Q

Glutamate is responsible for the synthesis of

A
  1. GABA (B6)

2. Glutathione

57
Q

Tyrosine synthesis

A

Phenylalamine to tyrosine (Phenylalamine hydroxylase and BH4)

58
Q

Aminoacid responsible for thyroxin synthesis

A

Tyrosine (tyrosine is synthesized by phenylalamine and BH4)

59
Q

Tyrosine (and phenylalamine) is responsible for the synthesis of

A
  1. Thyroxine 2. Dopa 3. Melanin 4. Dopamine. 5. NE 6. Epi
60
Q

Tyrosine to DOPA (enzyme and cofactor)

A

Tyrosine hydroxylase

BH4

61
Q

DOPA

A

Dihydrixyphenylalamine

62
Q

DOPA to melanin

A

Tyrosinase

63
Q

Tyrosinase deficiency

64
Q

Phenylalanine deficiency

A

Phenylketonurua

65
Q

DOPA decarboxylase inhibitor

66
Q

DOPA to Dopamine

A

DOPA decarboxylase

Vit B6

67
Q

Dopamine to Norepinephrine

A

Dopamine β hydroxylase

Vitamin C

68
Q

Dopamine metabolite

A

Homovallinic acid

69
Q

Norepinephrine metabolite

A

Normetanephrine to vanillymandelic acid

70
Q

Norepinephrine to epinephrine

71
Q

SAM (norepinephrine to epinephrine) regulation

A

Cortisol +

72
Q

Epinephrine metabolites

A

Metamephrine to vanillylmandenic acid

73
Q

Tyrosine, except dopamine, can be converted to

A

Homogentisic

74
Q

Homogentisic to maleyacetoacetic (TCA cycle as fumarate)

A

Homogenstisate oxidase

75
Q

Homogepentisate oxidase deficiency

A

Alkaptonuria

76
Q

BH4

A

Tetrahydrobiopterin

77
Q

Phenylketonuria pathophysiology

A
  1. Phenylalamine hydroxylase deficiency

2. Tetrahydrobiopterin deficiency (BH4) (malignant)

78
Q

PKU

A

Phenylketonuria

79
Q

Tyrosine/PKU/diet

A

Tyrosine becomes essential

80
Q

PKU/urine

A

Increased phenylketones in urine

81
Q

PKU symptoms

A
  1. Intellectual disability 2. Growth retardation 3. Seizures 4. Fair skin
  2. Eczema 6. Musty body odor
82
Q

PKU treatment

A
  • Decrease phenylalamine and increased tyrosine in diet
  • avoid artificial sweetener aspartame which contain phenylamines
  • Tetrahydrobipterin supplementation (BH4)
83
Q

PKU mode of inheritance

A

AR

Pleiotropy

84
Q

Maternal PKU

A

Lack of proper dietary therapy during pregnancy. Infant with microcephal, intellectual disability, growth reterdation, congenital heart defects

85
Q

PKU screening

A

2-3 days after birth (normal at birth because of maternal enzyme during fetal life)

86
Q

Phenylketones

A

Phenylacetate
Phenyllactate
Phenylpyruvate

87
Q

Disorder of aromatic amino acid metabolism

A

Musty body odor

89
Q

PKU patients must avoid

A

artificial sweetener aspartame which contain phenylamines

89
Q

Alkaptonuria - pathophysiology

A

Congenital deficiency of homogentisate oxidase in the degradative pathway of tyrosine to fumarate –> pigment-forming homogentistic acid accumulates in tissue

90
Q

Alkaptonuria mode of inheritance

92
Q

Alkaptonuria is a benign or a malignant disease

92
Q

Phenylketonuria incidence

93
Q

Alkaptonuria findings

A
  1. Dark connective tissue and sclerae (ochronosis)
  2. Urine turns black on prolonged exposure to air
  3. Debilitating arthralgias (Homogentisic acid toxic to cartilage)
94
Q

Homocystinuria mode of inheritance

95
Q

Homocystinuria results in excess

A

Homocysteine

96
Q

Niacin synthesis (amino acid and vitamin)

A

Tryptophan to niacin (B2, B6)

97
Q

Homocystinuria findings

A
  1. Increased homocysteine in urine 2. Intellectual disability 3. Osteoporosis 4. marfanoind habitus 5. Kyphosis 6. Lens subluxation (downward and inward). 7. Thrombosis. 8. Atherosclerosis (stroke and MI)
98
Q

Cysteine synthesis

A

Homocysteine + serine to cystathionine (cystathionine synthase +B6)
Cystathionine to cysteine

99
Q

Methionine synthesis

A

Homocysteine to methionine (methionine synthase +B12)

100
Q

Cystathionine synthase deficiency treatment

A

Low Methionine, increase cysteine and B12 and folate in diet

101
Q

Homocysteine methyltransferase (methionine synthase) deficiency - treatment

A

Increased methionine in diet

102
Q

Low affinity of Cystathionine synthase for pyridoxal phosphate treatment

A

Increased B6 and cysteine in diet

103
Q

Amino acids affected in cystinuria

A

COLA (mnemonic)

Cysteine, ornithine, lysine, arginine

104
Q

Cystinuria

A

Hedereditary defect of renal proximal convoluted tubular and intestinal amino acids trasnporter for Cysteine, Ornithine, Lysine, Argnine (COLA)

105
Q

Excess cysteine in urine

A

Precipitation of hexagonal cysteine stones

106
Q

Cystinuria mode of inheritance

107
Q

Cystinuria frequency

108
Q

What is cystine

A

2 cysteines commected by a disulfide bond

109
Q

Cystinuria diagnosis

A

Urinary cyanide-nitroprusside test

110
Q

Cystinuria treatment

A
  1. Urinary alkalization (eg.potasium citrate, acetalozamide)
  2. Chelating agents solubility of cystine stones
  3. Good hydration
111
Q

Homocystinuria types and mode of inheritance

A
  1. Cystathione synthase deficiency
  2. Low affinity of cystathione synthase for pyridoxal phosphate
  3. methione synthase deficiency
    ALL AR
112
Q

Maple syrup urine disease pathophysiology

A

Blocked degradation of branched amino acids (isoleucine, leucine, valine) due to decreased a-ketoacid dehydrogenase (B1).

113
Q

Branched amino acids

A
  1. Isoleucine
  2. Leucine
  3. Valine
114
Q

Maple syrup urine disease lab

A

Increased a ketoacids in the blood, especially those of leucine

115
Q

Maple syrup urine disease mode of inheritance

116
Q

Maple syrup urine disease complication

A
  1. Severe CNS defects
  2. Intellectual
  3. Death
117
Q

Maple syrup urine disease treatment

A

Restriction of lucine, isoleucine, valine in diet

Thiamine supplement

119
Q

Marple syrup urine presentation

A
  1. Urine smells like maple syrup/burnt sugar
  2. poor feeding
  3. vomiting
120
Q

homocysteine methyltransferase is AKA

A

homocysteine methyltransferase

Biochemistry FA (16 decks)

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