THE UREA CYCLE
G.H. MSONI
• the breakdown of macromolecules in the body
such as proteins, purines, pyrimidines and other
amino group containing molecules generates
ammonia(NH3)
• Ammonia can further be converted to
ammonium(NH4) which is a CNS irritant
• Accumulation of ammonia and ammonium in
the body is detrimental as these products are
toxic and death usually results.
• Organisms have developed different
mechanisms to get rid of ammonia and
ammonium by converting it to less toxic
substances that the body can tolerate
• Birds and other reptiles get rid of ammonia by
converting it to uric acid which is passed in
stool and are thus called URICOTERIC animals
• Fish and other reptiles directly get rid of
ammonia through their gills and are thus
called AMMONOTERIC animals
• Humans and other mammals get rid of
ammonia by converting it to urea which is less
toxic and is excreted via the kidneys and are
thus called UREOTERIC
• The urea cycle in humans and other mammals
occurs in the liver and to a less extent in the
kidneys
• It occurs in two sites of the cell that is the
mitochondrion matrix and the cytoplasm
• The urea cycle involves the use of rare amino
acids ornithine, citruline and Arginosuccinate
• The overall reaction involves the input of 3ATPs
two amino groups to synthesise urea
• The urea cycle is an amphibolic pathway
PATHWAY
Rxns of the urea cycle
• Step 1 ammonia from metabolism reacts with
bicarbonate(HCO3) under the influence of carbomyl
phosphate synthase to form carbomyl phosphate
• Step 2 Carbomyl phosphate then reacts with
ornithine to form citruline
• Step 3 citruline then leaves the mitochondrion into
the cytoplasm where it reacts with aspartic acid
which donates the second amino group to form
Arginosuccinate
• This is the second rxn at which ATP is utilised
this rxn is catalysed by Arginosuccinate synthase
• Step 4 is a cleavage rxn catalysed by
Arginosuccinate lyase to form arginine and
fumarate
• Step 5 is the urea production step catalysed by
arginase to form urea and ornithine which is
taken back to the mitochondrion to continue
the cycle

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THE UREA CYCLE lecture outline sem.pptx

  • 2. • the breakdown of macromolecules in the body such as proteins, purines, pyrimidines and other amino group containing molecules generates ammonia(NH3) • Ammonia can further be converted to ammonium(NH4) which is a CNS irritant • Accumulation of ammonia and ammonium in the body is detrimental as these products are toxic and death usually results.
  • 3. • Organisms have developed different mechanisms to get rid of ammonia and ammonium by converting it to less toxic substances that the body can tolerate • Birds and other reptiles get rid of ammonia by converting it to uric acid which is passed in stool and are thus called URICOTERIC animals
  • 4. • Fish and other reptiles directly get rid of ammonia through their gills and are thus called AMMONOTERIC animals • Humans and other mammals get rid of ammonia by converting it to urea which is less toxic and is excreted via the kidneys and are thus called UREOTERIC
  • 5. • The urea cycle in humans and other mammals occurs in the liver and to a less extent in the kidneys • It occurs in two sites of the cell that is the mitochondrion matrix and the cytoplasm • The urea cycle involves the use of rare amino acids ornithine, citruline and Arginosuccinate • The overall reaction involves the input of 3ATPs two amino groups to synthesise urea
  • 6. • The urea cycle is an amphibolic pathway
  • 8. Rxns of the urea cycle • Step 1 ammonia from metabolism reacts with bicarbonate(HCO3) under the influence of carbomyl phosphate synthase to form carbomyl phosphate • Step 2 Carbomyl phosphate then reacts with ornithine to form citruline • Step 3 citruline then leaves the mitochondrion into the cytoplasm where it reacts with aspartic acid which donates the second amino group to form Arginosuccinate
  • 9. • This is the second rxn at which ATP is utilised this rxn is catalysed by Arginosuccinate synthase • Step 4 is a cleavage rxn catalysed by Arginosuccinate lyase to form arginine and fumarate • Step 5 is the urea production step catalysed by arginase to form urea and ornithine which is taken back to the mitochondrion to continue the cycle