Urea cycle Flashcards

1
Q

urea cycle

in order

A
  1. NH3 + CO2
  2. Carbamoyl Phosphate (+Ornithine)
  3. Citrulline (+Aspartate, ATP)
  4. Argininosuccinate
  5. Arginine (,fumarate)
  6. Ornithine (,Urea)
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2
Q

urea cycle catalysts

in order

A
  1. CPS1
  2. Ornithine Transcarbamylase
  3. Argininosuccinate Synthesase
  4. Arganinosuccinase
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3
Q

site of occurence of urea cycle

A
  1. (hepatic) mitochondria
  2. cytoplasm
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4
Q

urea cycle

process in mitochondria

A
  1. NH2+CO2
  2. CPS1
  3. CP
  4. Ornithine
  5. Ornithine Transcarbamoyl
  6. Citrulline
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5
Q

rate limiting enzyme

A

Carbamoyl Phosphate Synthesase1 (CPS1)

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6
Q

Principle of:

Ornithine Transcarbamylase defficiency

A
  • Ornithine Transcarbamylase
  • Citrulline
  • Carbamoyl phosphate
  • orotic acid
  • ammonia
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