 Heme synthesis occurs : mitochondria and

cytoplasm

 First process and final step : mitochondria

 Intermediate : cytoplasm
TCA Tricarboxyllic Acid Cycle) Amino acid pool

Succynil coA + Glycine

ALA synthase Cofactor: vit.B6
(pyridoxal phosphate)

delta-aminolevulinic acid
(ALA)
 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)
Uroporphyrinogen decarboxcylase
Uroporphyrinogen III cosynthase
ALA dehydratase
Uroporpyrinogen III Coproporphyrinogen III
Porphobilinogen
Uroporpyrinogen I Coproporphyrinogen I
Uroporphyrinogen I synthase
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
 Heme

Ferrochelatase Fe2+

Protoporphyrin IX
proporphyrinogen IX oxydase

Protoporphyrinogen IX
Coproporphyrinogen III oxydase

Coproporphyrinogen III