Nitrogen Metabolism And Urea Cycle Flashcards
What is the rate limiting enzyme in urea cycle ?
Carbomoyl phosphate Synthase 1
What Activates CPS- 1 in urea cycle ?
NAG
Most common deficiency in Urea cycle ?
Orninthine transCARBOXYLASE def
XR
Increases Carbomoyl phosphate
Increased Ortic Acid
CF
Slurred speech
Vomit
Confusion
What does Transaminase do in nitrogen Metabolism ?
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)
What is the deficiency caused by Niacin B3?
HARTNUP
What are the CF of HARTNUP?
Neutral aromatic amino acid in urine
Skin leison
Loose stools
What are the CF of Hyperammonia?
Asterix
Slurr speech
Vomit
Blur vision
Less Alpha keto glutarate
Treatment : Limit protien in diet
What is the pneumonic of Urea cycle ?
Ordinary Caress Crappers are also furious about Urination
Orinthinie
Carbomoyl phosphate
Citrulline +Aspartate
Arginosuccinate
Fumarate +arginine
Urea