Nitrogen Metabolism Flashcards

1
Q

What are struvite crystals related to

A

Bacterial infections

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

What is calcium oxalate related to

A

Hyperparathyroidsim

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

What is the first step in nitrogen removal

A

Oxidative deamination

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

What holds the nitrogens to get removed

A

Glutamate

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

what is the carbon source for transamination reactions

A

Aspartate

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

What is the rate limiting step of urea cycle

A

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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

Where is nitrogen removed in the kidney

A

PCT

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

Hartnup and cystinuria are diseases related to ….

A

H- reuptake of nonpolar AA

C- reuptake of dibasic AA

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

Why are Hartnup and Cystinuria double whammy effects

A

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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

What is the MoA of Hartnup and Cystinuria

A

Deficient membrane transporters

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

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

A

Trp

It is used for NT and niacin (energy production)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

What is a classic symptom of hartnup

A

Pellagra-like dermatosis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

What causes a Hartnup event

A

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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

What is the treatment for hartnup disease

A

Niacin repletion and includes a high-protein diet

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

What is niacin or B3 also called

A

Nicotinic acid

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

What tissues need niacin more

A

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

17
Q

What are the dibasic AA

A
COAL
Cystine
Ornithine
Arginine 
Lysine
18
Q

What are the symptoms of cystinuria

A

Renal colic related to the stones

19
Q

How do we diagnose cystinuria

A

Nitroprusside test

20
Q

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

A

PEPT1 can take up peptides

21
Q

Secondary PKU is an issue with what enzyme

A

Dihydrobiopterin reductase

22
Q

What enzyme is affected in classic PKU

A

Phenylalanine hydroxylase

23
Q

What defective enzyme is responsible for albinism

A

Tyrosinase

Tyrosine—> melanin

24
Q

What is the most common IEM

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
31
What enzyme is related to type 2 tyrosinemia
Tyrosine aminotransferase
32
What is the inheritance of alkaptonuria
Autosomal recessive
33
What do we find with alkaptonuria
Black intervertebral disks and urine
34
What triad is associated with alkaptonuria
Homogenistic aciduria, ochronosis, and arthritis