Nitrogen Metabolism And Urea Cycle

Question 1

What is the rate limiting enzyme in urea cycle ?

Answer 1

Carbomoyl phosphate Synthase 1

Question 2

What Activates CPS- 1 in urea cycle ?

Answer 2

NAG

Question 3

Most common deficiency in Urea cycle ?

Answer 3

Orninthine transCARBOXYLASE def
XR
Increases Carbomoyl phosphate
Increased Ortic Acid

CF
Slurred speech
Vomit
Confusion

Question 4

What does Transaminase do in nitrogen Metabolism ?

Answer 4

Transfer Urea from Alanine or Aspartate to Alpha keto glutarate and back to the amino acid with the release of NH4+

in presence of B6 (PYRIDOXINE)

Question 5

What is the deficiency caused by Niacin B3?

Answer 5

HARTNUP

Question 6

What are the CF of HARTNUP?

Answer 6

Neutral aromatic amino acid in urine
Skin leison
Loose stools

Question 7

What are the CF of Hyperammonia?

Answer 7

Asterix
Slurr speech
Vomit
Blur vision
Less Alpha keto glutarate

Treatment : Limit protien in diet

Question 8

What is the pneumonic of Urea cycle ?

Answer 8

Ordinary Caress Crappers are also furious about Urination

Orinthinie
Carbomoyl phosphate
Citrulline +Aspartate
Arginosuccinate
Fumarate +arginine
Urea