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Renal > Nitrogen Metabolism > Flashcards

Nitrogen Metabolism Flashcards

1
Q

5 normal forms of nitrogen excretion

A 
Urea
Uric acid
Ammonia
Urobilinogen
Creatinine
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2
Q

Where does creatinine come from?

A

Breakdown of creatine phosphate in muscles

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3
Q

Where does uric acid come from?

Syndrome w/ uric acid build-up?

Causes what?

A

Purine breakdown

Lesch-Nyhan Syndrome

Gout, hyperuricosuria, hyperuricemia

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4
Q

Reasoning behind ammonia poisoning

A

Excess ammonia in the blood uses up alpha-KG and ATP to get rid of it, thus taking away from the TCA cycle and compromising energy source for the brain

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5
Q

Describe the general amino acid pathway through the body

A

Absorbed by GI mucosa or synthesized de novo

Put into the general A.A. pool

Used in protein build-up/break down OR degraded

Used as glucogenic/ketogenic building blocks OR put into the urine

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6
Q

Amino acids put into the urine get there via what forms?

A

Ammonia –> urea

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7
Q

Hartnup Disease

A

Defective neutral non-polar A.A. transporter (mucosa and tubules)

Very low levels of tryptophan, etc in the body

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8
Q

Cysteinuria

Amino acids affected?

Causes what?

A

Defective dibasic amino acid transporter

Cys, Lys, Arg

Crystal formation (stones)

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9
Q

Amino acids that require THF for metabolism

A

Methionine ONLY

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10
Q

Amino acids that require THB for metabolism

A

Phe, Tyr, Trp

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11
Q

What are the branched chain amino acids?

A

Valine
Isoleucine
Leucine

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12
Q

What disease is associated with the inability to digest branched chain AA’s?

Enzyme?

A

Maple Syrup Urine Disease

Alpha keto acid dehydrogenase

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13
Q

What is the common product of the breakdown of Met and branched-chain amino acids?

Significance?

A

Succinyl CoA

TCA cycle intermediate
Heme metabolism

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14
Q

A patient has Homocysteinuria. What is the patient at risk for?

What processes will be affected?

A

Atherosclerosis

TCA cycle and heme metabolism

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15
Q

Why do exercisers supplement with branched chain amino acids?

A

Succinyl CoA is the byproduct, thus more of it will increase TCA cycle ability and thus energy creation

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16
Q

What is the first intermediate of Met breakdown?

A

SAM

17
Q

What is THF needed for in Methionine metabolism?

What else is needed?

A

Recycling of Homocysteine into Methionine

B6, B12

18
Q

What are the co-enzymes needed for breakdown of branched chain amino acids?

A

Thiamine, CoA, FAD, lipoic acid, NAD

19
Q

A baby patient has urine that smells of burnt sugar, like syrup. It’s found that the cause is simply a deficiency of a co-factor. What is the co-factor?

What is the enzyme?

A

Thiamine (TPP)

Alpha keto acid dehydrogenase

20
Q

What are the 3 diseases to worry about with Phe metabolism?

A

PKU
Alkaptonuria
Tyrosinemia type 1

21
Q

What does Phe need for conversion to Tyr?

A

THB

22
Q

A patient is diagnosed with secondary Phenylketonuria (PKU). What is the cause of the problem?

What is the result?

A

Deficiency in THB, thus can’t convert Phe to Tyr for metabolism

Excess Phe converted to phenyl ketones, which are found in the urine

23
Q

A baby patient is diagnosed with Alkaptonuria. What is the result of this?

How is this typically diagnosed?

A

Homogentisate builds up, thus converts into alkaptones

Urine is blue/black due to alkaptones in the urine

24
Q

What is the end product of Phe metabolism?

Significance?

A

Fumarate

Another TCA intermediate, thus contributing to energy creation

25
Q

A patient is diagnosed with Tyrosinemia type 1. What is the result?

Danger?

A

Build-up of fumarylacetoacetate

Excess converts to succinyl acetone

Extremely toxic to liver and kidneys

26
Q

A patient has a deficiency in THB. What is the likely cause?

What metabolic issues does this cause?

A

Purine metabolism is impaired, thus THB is not created

Phe, Trp, and Tyr metabolism is also impaired

27
Q

A patient has Hartnup Disease. What substances will NOT be able to be made as well as a result?

Why?

A

NAD/NADP
Serotonin
Melatonin

Made via metabolism of tryptophan, which is low in serum

28
Q

How is melatonin made in amino acid metabolism?

Enzyme?

A

Trp –> serotonin –> melatonin

MAO (2nd step)

29
Q

What co-enzyme is produced from tryptophan?

Intermediate?

Needs what?

A

NAD+/NADP+

Niacin

Vitamin B6

30
Q

Breakdown pathway of Tyr

A 
Tyr
Dopa
Dopamine
NE
Epinephrine
31
Q

What all can be made from tyrosine?

A

Catecholamines
Melanin
Thyroid hormones

32
Q

A patient has a pheochromocytoma. What is likely to be found in the urine?

Why?

A

Homovanillic acid (HVA)

Vanillylmandelic acid (VMA)

Breakdown products of dopamine and norepinephrine, respectively

33
Q

What is MAO needed for in amino acid breakdown?

COMT?

A

Serotonin, dopamine, NE, Epi metabolisms

All those minus serotonin

34
Q

In what condition is uric acid most dangerous?

Why?

A

In acidic urine of distal tubule and cause kidney stones

Most likely to crystalize in acidic environment

35
Q

How is urea actually formed?

Precursor needed to drive the cycle?

A

Released from the conversion of Arg –> ornithine (using H2O) in the urea cycle

Carbamoyl phosphate

36
Q

What 2 things require carbamoyl phosphate?

A

Drive the urea cycle

Pyrimidine synthesis

37
Q

What two syndromes are important in terms of heme degradation?

A

Gilbert syndrome

Cholestasis

38
Q

A patient has lots of conjugated bilirubin in his urine. What is the name of the disease?

What does the urine look like?

A

Cholestasis

Very dark yellow

39
Q

A patient has trouble conjugating bilirubin, thus he has a build-up of unconjugated bilirubin. What is the disease called?

A

Gilbert Syndrome

Renal (11 decks)

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