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UWorld Review > AA > Flashcards

AA Flashcards

1
Q

How many essential AA are there

A

10

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2
Q

What are the essential AA

A

“PVT TIM HaLL”

  • Phe
  • Val
  • Trp
  • Thr
  • Ile
  • Met
  • His
  • Leu
  • Lys
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3
Q

Which AA are glucogenic

A
  • Met
  • His
  • Val
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4
Q

Which AA are glucogenic/ketogenic

A
  • Ile
  • Phe
  • Thr
  • Tryp
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5
Q

Which AA are purely ketogenic

A
  • Leu
  • Lys
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6
Q

Which AA are required during periods of growth

A

Arginine and Histidine

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7
Q

Which AA are increased in histones

A

Arginine and lysine

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8
Q

What AA makes tyrosine, dopamine, NE, Epi

A

Phe

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9
Q

Melanin comes from

A

Dopa (from tyrosine, from Phe)

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10
Q

Which AA makes NAD+/NADP+ and melatonin

A

Tryptophan

  • Niacin –> NAD+/NADP+
  • Serotonin –> Melatonin
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11
Q

Which AA makes heme

A

Glycine

Makes prophyrin (w/B6) which makes heme

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12
Q

Which AA makes GABA (w/B6) and Glutathione

A

Glutamate

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13
Q

Which AA makes creatine, urea, nitric oxide

A

Arginine

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14
Q

BH4 (tetrahydrobiopterin) is required for what processes

A
  1. Phe to Tyrosine
  2. Tyrosine to Dopa
  3. Tryptophan to serotonin
  4. Arginine to Nitric oxide
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15
Q

B6 is required for which AA processes

A
  1. Dopa to dopamine
  2. Tryptophan to Niacin (w/B2)
  3. Tryptophan to serotonin (w/BH4)
  4. Histidine to Histamine
  5. Glycine to Porphyrin
  6. Glutamate to GABA
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16
Q

Vitamin C is required for what AA process

A

Dopamine to NE

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17
Q

SAM is required for what AA process

A

NE to Epi

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18
Q

What are the steps to making catecholamines

A

Phe–>Tyrosine–>DOPA–>Dopamine–>NE–>Epi

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19
Q

What is defective in PKU

A

phenylalanine hydroxylase

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20
Q

What is defective in Alkaptonuria

A

Homogentisate oxidase

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21
Q

What is defective in albinism

A

Tyrosinase

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22
Q

Carpidopa inhibits what

A

DOPA decarboxylse

no DOPA to Dopamine

23
Q

Cortisol acitvates

A

phenylethanolamine-N-methyltransferase

NE to Epi

24
Q

Epi is turned into Metanepherine by

A

COMT

25
Q

NE is turned into normetanephrine (vanillylmandelic acid) by

A

COMT

26
Q

PKU is d/t

A

decrease Phe (aromatic AA) hydroxylase or decrease BH4 cofactor (malignant PKU)

AR

screening 2-3 days after birth

27
Q

What becomes essential in PKU

A

Tyrosine

28
Q

Increase in Phe causes

A

increase in phenyl ketones in urine

29
Q

Symptoms of PKU

A
  • intellectual disability
  • growth retardation
  • seizures
  • fair complexion
  • eczema
  • musty body odor
30
Q

How to treat PKU

A
  • decrease Phe
  • increase tyrosine in diet
  • BH4 supplementation
31
Q

Why are PKU babies normal at birth

A

maternal enzyme present during fetal life

32
Q

What must PKU patients avoid

A

artificial sweetener aspartame

has Phe

33
Q

Maple syrup urine disease is d/t

A

AR: blocked degradation of branched AA d/t decreased branched chain alpha-ketoacid DH (B1)

  • Ile
  • leu
  • val
34
Q

In maple syrup disease there is an increase in what

A

alpha-ketoacids in blood, especially those of leucine

35
Q

How to treat maple syrup disease

A
  • restrict branched chain AA (val, ile, leu) in diet
  • thiamine supplementation
36
Q

Maple syrup disease symptoms

A
  • urine smells like burnt sugar/maple syrup
  • vomiting
  • poor feeding
  • severe CNS defects
  • interllectual disability
  • death
37
Q

Alkaptonuria is d/t

A

AR congenital deficiency of homogentisate oxidase in degradative path of tyrosine to fumarate

  • pigment forming homogentisic acid builds up in tissue
  • usually benign
38
Q

Symptoms of alkaptonuria

A
  • bluish-black connective tissue, ear cartilage, sclerae (ochronosis)
  • urine turns black on prolonged exposure to air
  • possible debiliating arthalgias (homogentisic acid toxic to cartilage)
39
Q

Homocystinuria is d/t what 4 causes

A

AR

  1. cystathionine deficiency
  2. decreased affinity of cystathionine synthase for pyridoxal phosphate
  3. methionine synthase deficiency
  4. MTHFR deficiency
40
Q

How to treat homocystinuria d/t cystathionine synthase deficiency

A
  1. decrease methionine
  2. increase cysteine, B6, B12, folate in diet
41
Q

How to treat homocystinuria d/t decrease affinity of cystathionine synthase for pyridoxal phosphate

A

increase B6 (alot) and cysteine in diet

42
Q

How to treat homocystinuria d/t methionine synthase deficiency

A

increase methionine in diet

43
Q

How to treat homocystinuria d/t MTHFR deficiency

A

increase folate in diet

44
Q

All forms of homocystinuria cause

A
  1. increase homocysteine in urine
  2. osteoporsis
  3. marfanoid habitus (binds to fibrillin-1)
  4. ocular changes (down and in lens subluxation)
  5. cardio effects (thrombosis and atherosclerosis, stroke and MI)
  6. Kyphosis
  7. intellectual disability
  8. fair complexion
45
Q

Why does homocystinuria cause thrombosis

A

homocysteine binds to endothelial cells = proinfammatory cytokines

46
Q

Cystinuria is d/t

A

hereditary defect of renal PCT and intestinal AA transporter that prevents reabsorption of “COLA”

  • cystine
  • ornithine
  • lysine
  • arginine
47
Q

what can too much cystine in urine do

A

hexagonal cystine stones

48
Q

how to treat cystinuria

A
  • urinary alkalinization (potassium citrate, acetazolamide)
  • chelating agents (penicillamine) increase solubility of stones
  • good hydration
49
Q

How to diagnose cystinuria

A

AR

urinary cyanide-nitroprusside test

50
Q

What is propionic acidemia d/t

A

AR deficiency of propionyl-CoA carboxylase

excess propionyl-CoA

decrease methymalonic acid

51
Q

findings of propionic acidemia

A
  • poor feeding
  • vomiting
  • hypotonia
  • anion gap metabolic acidosis
  • hepatomegaly
  • seizures
52
Q

How to treat propionic acidemia

A

low protein diet that does not have ile, met, thr, val

53
Q

Substances that metabolize into propionyl-CoA cause you to VOMIT

A

Valine

Odd-chain FA

Methionine

Isoleucine

Threonine

UWorld Review (35 decks)

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