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 Heme synthesis occurs : mitochondria and
cytoplasm
 First process and final step : mitochondria
 Intermediate : cytoplasm
TCA Tricarboxyllic Acid Cycle)
Succynil coA + Glycine
delta-aminolevulinic acid
(ALA)
Amino acid pool
Cofactor: vit.B6
(pyridoxal phosphate)
ALA synthase
 Pirydoxal Phosphate antagonists :
◦ Penicilline
◦ Isoniazide
 ALA synthase is stimulated by :
◦ Barbiturates
◦ Steorids such as testosteron and oral contraceptives
2 molecules of delta-aminolevulinic acid (ALA)
Porphobilinogen
Uroporpyrinogen III
Uroporpyrinogen I
Coproporphyrinogen III
Coproporphyrinogen I
ALA dehydratase
Uroporphyrinogen III cosynthase
Uroporphyrinogen I synthase
Uroporphyrinogen decarboxcylase
Uroporphyrinogen decarboxcylase
Pyrrole
(Ring)
 ALA dehydratase = Porphobilinogen (PBG)
Synthase
 -SH containing enzyme
 Very susceptible to inhibition by heavy
metals, especially leads (Pb2+)
 Increased ALA in blood  detected increasing
in urine  lead poisoning  toxic to brain 
affected nervous system
autoxidation
Sulfides,
selenides,
tellurides, etc
Coproporphyrinogen III
Protoporphyrinogen IX
Protoporphyrin IX
Heme
Coproporphyrinogen III oxydase
proporphyrinogen IX oxydase
Ferrochelatase Fe2+
kp 1.4.2.7 Heme Synthesis.ppt
kp 1.4.2.7 Heme Synthesis.ppt

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kp 1.4.2.7 Heme Synthesis.ppt

  • 1.
  • 2.  Heme synthesis occurs : mitochondria and cytoplasm  First process and final step : mitochondria  Intermediate : cytoplasm
  • 3.
  • 4. TCA Tricarboxyllic Acid Cycle) Succynil coA + Glycine delta-aminolevulinic acid (ALA) Amino acid pool Cofactor: vit.B6 (pyridoxal phosphate) ALA synthase
  • 5.
  • 6.  Pirydoxal Phosphate antagonists : ◦ Penicilline ◦ Isoniazide  ALA synthase is stimulated by : ◦ Barbiturates ◦ Steorids such as testosteron and oral contraceptives
  • 7. 2 molecules of delta-aminolevulinic acid (ALA) Porphobilinogen Uroporpyrinogen III Uroporpyrinogen I Coproporphyrinogen III Coproporphyrinogen I ALA dehydratase Uroporphyrinogen III cosynthase Uroporphyrinogen I synthase Uroporphyrinogen decarboxcylase Uroporphyrinogen decarboxcylase
  • 9.  ALA dehydratase = Porphobilinogen (PBG) Synthase  -SH containing enzyme  Very susceptible to inhibition by heavy metals, especially leads (Pb2+)  Increased ALA in blood  detected increasing in urine  lead poisoning  toxic to brain  affected nervous system
  • 11.
  • 12. Coproporphyrinogen III Protoporphyrinogen IX Protoporphyrin IX Heme Coproporphyrinogen III oxydase proporphyrinogen IX oxydase Ferrochelatase Fe2+