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Biochemistry FA > Amino Acids > Flashcards

Amino Acids Flashcards

(120 cards)

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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
Urea is excreted by
Kidney
25
Urea cycle site
Both Mitochondria and cytoplasm of the liver
26
Enzyme responsible for urea production
Arginase
27
Aminoacids(NH3) to a-ketoacids causes
A ketoglutarate to glutamate(NH3)
28
Glutamate(NH3) to a-ketoglutorate causes
Pyruvate to alanine (NH3)
29
Cori cycle
Glucose to pyruvate to lactate (muscle) Lactate to pyruvate to glucose (liver) Glucose back to muscle
30
Alanine cycle (cahill cycle)
Pyruvate-alanine (muscle) Alanine(NH3) to pyruvate (liver) converting a-ketoglutorate to glutamate(NH3) pyruvate to glucose (liver) Liver back to muscle
31
Hyperammonemia can be divided to
1. Acquired (liver disease) | 2. Hereditary (urea cycle enzyme deficiencies
32
Transport of ammonia by
Alanine and glutamate
33
Hyperammonemia results in
Excess NH4+
34
Excess NH4+ in hyperammonemia results in
a-ketoglutarate depletion, leading to inhibition of TCA cycle
35
Carbamoyl phosphate synthetase I cofactor
N-acteylglutammate
36
Hyperammonemia treatment
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
37
N-acetyglutamate deficiency leads to
Hyperammonemia
38
N-acetyglutamate deficiency is identical to
Carbamoyl synthetase I deficiency
39
Ornithine trancarbamylase deficiency mode of inheritance
XR (vs other urea cycle enzyme deficiency which are autosomal recessive)
40
Lab findings that suggest N-acetyglutamate deficiency and clinical presentation
Increased ornithine with normal urea cycle enzymes 1. poorly regulated respiration and body Q 2. poor feeding 3. developmental delay 4. intellectual disability
41
Most common urea cycle disorder
Ornithine trancarbamylase deficiency
42
Urea cycle enzymes deficiencies are AR except
Ornithine trancarbamylase deficiency --> XR
43
Ornithine trancarbamylase deficiency / pathophysiology
Interferes with the body's ability to eliminate ammonia
44
Ornithine trancarbamylase deficiency/ often evident in (age)
First few days of live, but may present with late onset
45
Ornithine trancarbamylase deficiency /what is happening with the excess carbamoyl phosphate
Is converted to orotic acid
46
Ornithine trancarbamylase deficiency findings
1. Increased orotic acid blood and urine 2. Decreased BUN 3. Symptoms of hyperammonemia 4. NO MEGALOBLASTIC ANEMIA (vs orotic aciduria)
47
Orotic aciduria vs Ornithine trancarbamylase deficiency (lab)
Ornithine trancarbamylase deficiency has no megaloblastic anemia
48
Amonoacid and vitamin to heme
Glycine to porphyrin (B6) | Porphyrin to heme
49
Amina acid responsible for heme synthesis | Vitamin responsible for heme synthesis
Glycine | B6
50
Histamini synthesis (amino acid and vitamin)
Histidine to histamine (B6)
51
Tryptophan participate in the synthesis of
Niacin (NAD, NADP) Serotonin Melatonin
52
Ammonia intoxication
1. Slurring of speech 2. Tremor (asterixis) 3. Somnolence 4. Vomiting 5. Cerebral edema 6. Blurring vision
53
Arginine is responsible for the synthesis of
1. Creatinine 2. Urea 3. Nitric oxide (BH4 as a cofactor)
54
Synthesis of melatonin (aminoacid and vitamin)
Tryptophan to serotonin (B6+BH4) | Serotonin to melatonin
55
Nitric oxide synthesis (amino acid and coafactor)
Arginine to nitric oxide (BH4)
56
Glutamate is responsible for the synthesis of
1. GABA (B6) | 2. Glutathione
57
Tyrosine synthesis
Phenylalamine to tyrosine (Phenylalamine hydroxylase and BH4)
58
Aminoacid responsible for thyroxin synthesis
Tyrosine (tyrosine is synthesized by phenylalamine and BH4)
59
Tyrosine (and phenylalamine) is responsible for the synthesis of
1. Thyroxine 2. Dopa 3. Melanin 4. Dopamine. 5. NE 6. Epi
60
Tyrosine to DOPA (enzyme and cofactor)
Tyrosine hydroxylase | BH4
61
DOPA
Dihydrixyphenylalamine
62
DOPA to melanin
Tyrosinase
63
Tyrosinase deficiency
Albinism
64
Phenylalanine deficiency
Phenylketonurua
65
DOPA decarboxylase inhibitor
Carbidopa
66
DOPA to Dopamine
DOPA decarboxylase | Vit B6
67
Dopamine to Norepinephrine
Dopamine β hydroxylase | Vitamin C
68
Dopamine metabolite
Homovallinic acid
69
Norepinephrine metabolite
Normetanephrine to vanillymandelic acid
70
Norepinephrine to epinephrine
SAM
71
SAM (norepinephrine to epinephrine) regulation
Cortisol +
72
Epinephrine metabolites
Metamephrine to vanillylmandenic acid
73
Tyrosine, except dopamine, can be converted to
Homogentisic
74
Homogentisic to maleyacetoacetic (TCA cycle as fumarate)
Homogenstisate oxidase
75
Homogepentisate oxidase deficiency
Alkaptonuria
76
BH4
Tetrahydrobiopterin
77
Phenylketonuria pathophysiology
1. Phenylalamine hydroxylase deficiency | 2. Tetrahydrobiopterin deficiency (BH4) (malignant)
78
PKU
Phenylketonuria
79
Tyrosine/PKU/diet
Tyrosine becomes essential
80
PKU/urine
Increased phenylketones in urine
81
PKU symptoms
1. Intellectual disability 2. Growth retardation 3. Seizures 4. Fair skin 5. Eczema 6. Musty body odor
82
PKU treatment
- Decrease phenylalamine and increased tyrosine in diet - avoid artificial sweetener aspartame which contain phenylamines - Tetrahydrobipterin supplementation (BH4)
83
PKU mode of inheritance
AR | Pleiotropy
84
Maternal PKU
Lack of proper dietary therapy during pregnancy. Infant with microcephal, intellectual disability, growth reterdation, congenital heart defects
85
PKU screening
2-3 days after birth (normal at birth because of maternal enzyme during fetal life)
86
Phenylketones
Phenylacetate Phenyllactate Phenylpyruvate
87
Disorder of aromatic amino acid metabolism
Musty body odor
89
PKU patients must avoid
artificial sweetener aspartame which contain phenylamines
89
Alkaptonuria - pathophysiology
Congenital deficiency of homogentisate oxidase in the degradative pathway of tyrosine to fumarate --> pigment-forming homogentistic acid accumulates in tissue
90
Alkaptonuria mode of inheritance
AR
92
Alkaptonuria is a benign or a malignant disease
Benign
92
Phenylketonuria incidence
1/10,000
93
Alkaptonuria findings
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
Homocystinuria mode of inheritance
AR
95
Homocystinuria results in excess
Homocysteine
96
Niacin synthesis (amino acid and vitamin)
Tryptophan to niacin (B2, B6)
97
Homocystinuria findings
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
Cysteine synthesis
Homocysteine + serine to cystathionine (cystathionine synthase +B6) Cystathionine to cysteine
99
Methionine synthesis
Homocysteine to methionine (methionine synthase +B12)
100
Cystathionine synthase deficiency treatment
Low Methionine, increase cysteine and B12 and folate in diet
101
Homocysteine methyltransferase (methionine synthase) deficiency - treatment
Increased methionine in diet
102
Low affinity of Cystathionine synthase for pyridoxal phosphate treatment
Increased B6 and cysteine in diet
103
Amino acids affected in cystinuria
COLA (mnemonic) | Cysteine, ornithine, lysine, arginine
104
Cystinuria
Hedereditary defect of renal proximal convoluted tubular and intestinal amino acids trasnporter for Cysteine, Ornithine, Lysine, Argnine (COLA)
105
Excess cysteine in urine
Precipitation of hexagonal cysteine stones
106
Cystinuria mode of inheritance
AR
107
Cystinuria frequency
1/7000
108
What is cystine
2 cysteines commected by a disulfide bond
109
Cystinuria diagnosis
Urinary cyanide-nitroprusside test
110
Cystinuria treatment
1. Urinary alkalization (eg.potasium citrate, acetalozamide) 2. Chelating agents solubility of cystine stones 3. Good hydration
111
Homocystinuria types and mode of inheritance
1. Cystathione synthase deficiency 2. Low affinity of cystathione synthase for pyridoxal phosphate 3. methione synthase deficiency ALL AR
112
Maple syrup urine disease pathophysiology
Blocked degradation of branched amino acids (isoleucine, leucine, valine) due to decreased a-ketoacid dehydrogenase (B1).
113
Branched amino acids
1. Isoleucine 2. Leucine 3. Valine
114
Maple syrup urine disease lab
Increased a ketoacids in the blood, especially those of leucine
115
Maple syrup urine disease mode of inheritance
AR
116
Maple syrup urine disease complication
1. Severe CNS defects 2. Intellectual 3. Death
117
Maple syrup urine disease treatment
Restriction of lucine, isoleucine, valine in diet | Thiamine supplement
119
Marple syrup urine presentation
1. Urine smells like maple syrup/burnt sugar 2. poor feeding 3. vomiting
120
homocysteine methyltransferase is AKA
homocysteine methyltransferase

Biochemistry FA (16 decks)

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