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Renal I > Nitrogen Metabolism - Seidler > Flashcards

Nitrogen Metabolism - Seidler Flashcards

1
Q

Amino acid pool

A

Population of free AA in the body

N released during AA degradation is converted to urea and excreted in urine

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2
Q

Hartnup disease

A

Tryptophan - defect in transporter

Sxs - nystagmus, FTT, intermittent ataxia

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3
Q

Cystinuria

A

Dimeric AA cysteine, Arg, Lys

Cystine crystals = stones in kidneys = renal colic

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4
Q

Glucogenic AA

A

18 of 20 AA

Can be degraded to pyruvate or intermediates of the TCA cycle

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5
Q

Keto acid dehydrogenase enzyme complexes

A

Branched chain a-keto acid dehydrogenates
Requires 5 cofactors - TPP, lipoic acid, CoA, FAD, NAD
Unidirectional reaction - E1, E2, E3

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6
Q

Maple syrup urine disease

A

Presence of branched chain AA and there a-keto derivatives in urine
Also accumulates in blood - brain problems

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7
Q

What is something that can sometimes fix maple syrup urine disease?

A

Thiamine supplementation

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8
Q

What can lead to secondary PKU?

A

Defects in biosynthesis or regeneration of tetrahydrobiopterin (THB)

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9
Q

PKU

A

Defects in activity of phenylalanine hydroxyls activity

Disrupts NT and blocks AA transport in brain and myelin

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10
Q

Tyrosinemias

A

3 types
Type I most common
Characterized by elevated blood tyrosine

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11
Q

Alkaptonuria

A

Defect in homogentisate oxidase - an enzyme in Tyr degradative pathway
Causes it to accumulate
Blue diaper

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12
Q

MAO and COMT

A

2 enzymes involved in degradation of dopamine into homovallic acid (HVA)
Also VMA
Both HVA and VMA in urine of pts

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13
Q

What is the toxic agent in ammonia toxicity?

A

Uncharged ammonia - NH3

Due to its ability to cross the membrane = edema

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14
Q

Gout

A

High levels of uric acid in blood due to overproduction
Lesch-Nyhan syndrome
Tx - colchicine

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15
Q

Hyperammonemia

A

Results from defects in any of 6 enzymes associated with urea cycle or 3 transporters
Tx - limit protein intake

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16
Q

Type I citrullinemia

A

Autosomal recessive

Def in arginosuccinate synthetase

17
Q

Type II citrullinemia

A

Less common in adults

Defect in transporter Citrin

18
Q

Unconjugated bilirubin

A

Insoluble in water - Corbert’s?

19
Q

Conjugated bilirubin

A

Soluble in water - found in urine

Cholestasis

20
Q

5 Normal nitrogen excretory compounds

A 
Urea
Uric acid
Ammonia
Urobilinogen
Creatinine
21
Q

Trp derivatives

A

Niacin

Serotonin –> Melatonin

22
Q

Tyr derivatives

A

Dopa
Dopamine
NE
Epinephrine

23
Q

SAM

A

functions as a methyl donor for various reactions

ex. NE to Epi

24
Q

Homocystinuria

A

Risk factor for atherosclerosis

25
Q

Excess ammonia removed as either

A

GLU or GLN

26
Q

Urobilin

A

Breakdown of urobilinogen

Gives urine yellow color

Renal I (20 decks)

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