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Flashcards in Nitrogen Metabolism Deck (34)
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1

What are struvite crystals related to

Bacterial infections

2

What is calcium oxalate related to

Hyperparathyroidsim

3

What is the first step in nitrogen removal

Oxidative deamination

4

What holds the nitrogens to get removed

Glutamate

5

what is the carbon source for transamination reactions

Aspartate

6

What is the rate limiting step of urea cycle

Conversion of ammonia and carbamoyl phosphate via carbamoyl phosphate synthetase and NAG activation

7

Where is nitrogen removed in the kidney

PCT

8

Hartnup and cystinuria are diseases related to ....

H- reuptake of nonpolar AA
C- reuptake of dibasic AA

9

Why are Hartnup and Cystinuria double whammy effects

Because they have both the reuptake issue in the kidney but they also have the uptake issue in the intestines

10

What is the MoA of Hartnup and Cystinuria

Deficient membrane transporters

11

Hartnup is devastating when looking at one particular AA, which one causes the most issues?

Trp

It is used for NT and niacin (energy production)

12

What is a classic symptom of hartnup

Pellagra-like dermatosis

13

What causes a Hartnup event

Sunlight, fever, drugs, emotional or physical stress, or poor nutrition

14

What is the treatment for hartnup disease

Niacin repletion and includes a high-protein diet

15

What is niacin or B3 also called

Nicotinic acid

16

What tissues need niacin more

Tissues that have high energy demands or tissues that have a high turnover rate

17

What are the dibasic AA

COAL
Cystine
Ornithine
Arginine
Lysine

18

What are the symptoms of cystinuria

Renal colic related to the stones

19

How do we diagnose cystinuria

Nitroprusside test

20

What is the transporter that can help with uptake/reuptake issues

PEPT1 can take up peptides

21

Secondary PKU is an issue with what enzyme

Dihydrobiopterin reductase

22

What enzyme is affected in classic PKU

Phenylalanine hydroxylase

23

What defective enzyme is responsible for albinism

Tyrosinase

Tyrosine—> melanin

24

What is the most common IEM

PKU

25

What are the symptoms of PKU? Treatment?

Musty odor in urine cause by phenylacetate (also blocks AA transport and neurotransmission in the brain)

Limit Phe and supplement Tyr

26

When is it critical to have diagnosed PKU

Before 2 weeks

27

What is the upper limit of Phe in the blood

6 mg/dL

28

What helps with PKU in diet

Treatment of tetrahydropiopterin (5-20mg/kg a day)which is a PAH cofactor

29

What enzyme is linked to type 1 tyrosinemia

Fumaraylacetate hydrolase

30

What is a main symptom of type 1 tyrosinemia

Cabbage smelling urine