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 (ORNITHINE CYCLE)
•THE UREA CYCLE OR ORNITHINE CYCLE CONVERTS EXCESS AMMONIA
INTO UREA IN THE MITOCHONDRIA OF LIVER CELLS.
•THE OVERALL REACTION FOR UREA FORMATION FROM AMMONIA IS AS
FOLLOWS:
2 AMMONIA + CO2 + 3ATP ---> UREA + WATER +3ADP
•THIS CYCLE WAS THE FIRST METABOLIC CYCLE TO BE DISCOVERED
(HANS KREBS AND KURT HENSELEIT, 1932), FIVE YEARS BEFORE THE
DISCOVERY OF THE TCA CYCLE.
•THE UREA CYCLE TAKES PLACE PRIMARILY IN THE LIVER AND, TO
A LESSER EXTENT, IN THE KIDNEYS.
 Urea
 UREA, ALSO KNOWN AS CARBAMIDE, IS AN ORGANIC COMPOUND WITH
CHEMICAL FORMULA CO(NH₂)₂. THIS AMIDE HAS TWO –NH₂ GROUPS
JOINED BY A CARBONYL FUNCTIONAL GROUP.
MOLAR MASS: 60.06 G/MOL
MELTING POINT: 133 °C
FORMULA: CH4N2O
SOLUBLE IN: WATER, GLYCEROL, ETHANOL
NORMAL BODY LEVEL:-20 MG/DL (2.5 TO 7.1 MMOL/L) IS CONSIDERED
NORMAL.
•Steps in the Urea Cycle
NH3 + CO2 + 2ATP → carbamoyl phosphate + 2ADP + Pi
•Carbamoyl Phosphate
•In the first step of the Krebs-Henseleit cycle
CARBAMOYL PHOSPHATE + ORNITHINE → CITRULLINE + PI
•THE SECOND STEP OF THE CYCLE:
CITRULLINE
ORNITHINE
CARBAMOYL PHOSPHATE
•THE THIRD STEP OF THE KREBS-HENSELEIT CYCLE
CITRULLINE + ATP + ASPARTATE → ARGININOSUCCINATE + AMP + PPI
Argininosuccinate → arginine + fumarate
Fumaric acid
•THE FOURTH STEP INVOLVES
ARGININOSUCCINATE
•IN THE FIFTH AND LAST STEP OF THE UREA CYCLE
ARGININE → UREA + ORNITHINE
ORNITHINE
UREA
IN THE FIRST STEP OF THE KREBS-HENSELEIT CYCLE, AMMONIA
PRODUCED IN THE MITOCHONDRIA IS CONVERTED TO CARBAMOYL
PHOSPHATE BY AN ENZYME CALLED CARBAMOYL PHOSPHATE SYNTHETASE I
THE SECOND STEP INVOLVES THE TRANSFER OF A CARBAMOYL GROUP
FROM CARBAMOYL PHOSPHATE TO ORNITHINE TO FORM CITRULLINE. THIS
STEP IS CATALYZED BY THE ENZYME ORNITHINE TRANSCARBAMOYLASE
(OTC) .
THE THIRD STEP IS CATALYZED BY AN ENZYME CALLED
ARGININOSUCCINATE SYNTHETASE, WHICH USES CITRULLINE AND ATP TO
FORM A CITRULLYL-AMP INTERMEDIATE, WHICH REACTS WITH AN AMINO
GROUP FROM ASPARTATE TO PRODUCE ARGININOSUCCINATE
KREBS-HENSELEIT CYCLE
THE FOURTH STEP INVOLVES THE CLEAVAGE OF ARGININOSUCCINATE
TO FORM FUMARATE AND ARGININE. ARGININOSUCCINATE LYASE IS
THE ENZYME CATALYZING THIS REACTION
IN THE FIFTH AND LAST STEP OF THE UREA CYCLE, ARGININE IS
HYDROLYZED TO FORM UREA AND ORNITHINE
THE OVERALL REACTION CAN BE GIVEN AS FOLLOWS:
2NH3 + CO2 + 3ATP G UREA + 2ADP + AMP + PPI + 2PI
KREBS-HENSELEIT CYCLE
urea cycle{ornithine cycle}

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urea cycle{ornithine cycle}

  • 1.  (ORNITHINE CYCLE) •THE UREA CYCLE OR ORNITHINE CYCLE CONVERTS EXCESS AMMONIA INTO UREA IN THE MITOCHONDRIA OF LIVER CELLS. •THE OVERALL REACTION FOR UREA FORMATION FROM AMMONIA IS AS FOLLOWS: 2 AMMONIA + CO2 + 3ATP ---> UREA + WATER +3ADP •THIS CYCLE WAS THE FIRST METABOLIC CYCLE TO BE DISCOVERED (HANS KREBS AND KURT HENSELEIT, 1932), FIVE YEARS BEFORE THE DISCOVERY OF THE TCA CYCLE. •THE UREA CYCLE TAKES PLACE PRIMARILY IN THE LIVER AND, TO A LESSER EXTENT, IN THE KIDNEYS.
  • 2.  Urea  UREA, ALSO KNOWN AS CARBAMIDE, IS AN ORGANIC COMPOUND WITH CHEMICAL FORMULA CO(NH₂)₂. THIS AMIDE HAS TWO –NH₂ GROUPS JOINED BY A CARBONYL FUNCTIONAL GROUP. MOLAR MASS: 60.06 G/MOL MELTING POINT: 133 °C FORMULA: CH4N2O SOLUBLE IN: WATER, GLYCEROL, ETHANOL NORMAL BODY LEVEL:-20 MG/DL (2.5 TO 7.1 MMOL/L) IS CONSIDERED NORMAL.
  • 3. •Steps in the Urea Cycle NH3 + CO2 + 2ATP → carbamoyl phosphate + 2ADP + Pi •Carbamoyl Phosphate •In the first step of the Krebs-Henseleit cycle
  • 4. CARBAMOYL PHOSPHATE + ORNITHINE → CITRULLINE + PI •THE SECOND STEP OF THE CYCLE: CITRULLINE ORNITHINE CARBAMOYL PHOSPHATE
  • 5. •THE THIRD STEP OF THE KREBS-HENSELEIT CYCLE CITRULLINE + ATP + ASPARTATE → ARGININOSUCCINATE + AMP + PPI
  • 6. Argininosuccinate → arginine + fumarate Fumaric acid •THE FOURTH STEP INVOLVES ARGININOSUCCINATE
  • 7. •IN THE FIFTH AND LAST STEP OF THE UREA CYCLE ARGININE → UREA + ORNITHINE ORNITHINE UREA
  • 8. IN THE FIRST STEP OF THE KREBS-HENSELEIT CYCLE, AMMONIA PRODUCED IN THE MITOCHONDRIA IS CONVERTED TO CARBAMOYL PHOSPHATE BY AN ENZYME CALLED CARBAMOYL PHOSPHATE SYNTHETASE I THE SECOND STEP INVOLVES THE TRANSFER OF A CARBAMOYL GROUP FROM CARBAMOYL PHOSPHATE TO ORNITHINE TO FORM CITRULLINE. THIS STEP IS CATALYZED BY THE ENZYME ORNITHINE TRANSCARBAMOYLASE (OTC) . THE THIRD STEP IS CATALYZED BY AN ENZYME CALLED ARGININOSUCCINATE SYNTHETASE, WHICH USES CITRULLINE AND ATP TO FORM A CITRULLYL-AMP INTERMEDIATE, WHICH REACTS WITH AN AMINO GROUP FROM ASPARTATE TO PRODUCE ARGININOSUCCINATE KREBS-HENSELEIT CYCLE
  • 9. THE FOURTH STEP INVOLVES THE CLEAVAGE OF ARGININOSUCCINATE TO FORM FUMARATE AND ARGININE. ARGININOSUCCINATE LYASE IS THE ENZYME CATALYZING THIS REACTION IN THE FIFTH AND LAST STEP OF THE UREA CYCLE, ARGININE IS HYDROLYZED TO FORM UREA AND ORNITHINE THE OVERALL REACTION CAN BE GIVEN AS FOLLOWS: 2NH3 + CO2 + 3ATP G UREA + 2ADP + AMP + PPI + 2PI