Nitrogen Metabolism And Urea Cycle Flashcards

1
Q

What is the rate limiting enzyme in urea cycle ?

A

Carbomoyl phosphate Synthase 1

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2
Q

What Activates CPS- 1 in urea cycle ?

A

NAG

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3
Q

Most common deficiency in Urea cycle ?

A

Orninthine transCARBOXYLASE def
XR
Increases Carbomoyl phosphate
Increased Ortic Acid

CF
Slurred speech
Vomit
Confusion

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4
Q

What does Transaminase do in nitrogen Metabolism ?

A

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)

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5
Q

What is the deficiency caused by Niacin B3?

A

HARTNUP

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6
Q

What are the CF of HARTNUP?

A

Neutral aromatic amino acid in urine
Skin leison
Loose stools

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7
Q

What are the CF of Hyperammonia?

A

Asterix
Slurr speech
Vomit
Blur vision
Less Alpha keto glutarate

Treatment : Limit protien in diet

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8
Q

What is the pneumonic of Urea cycle ?

A

Ordinary Caress Crappers are also furious about Urination

Orinthinie
Carbomoyl phosphate
Citrulline +Aspartate
Arginosuccinate
Fumarate +arginine
Urea

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