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8.metabolism of Heme
8.metabolism of Heme
8.metabolism of Heme
White
cell
serum
platelet
Blood
cell
Read
cell
globin
hemoglobin
heme
pyrrole rings
heme
porphobilinogen.
Formation of protoporphyrin IX
Clinical importance:
Ferrochelatase enzyme is inhibited by lead
Summary
① Major sites of synthesis is liver and bone marrow
(erythroblasts). Matured red blood cells have no
mitochondria, so can’t make heme.
porphobilinogen
FURTHER STEPS
Protoporphyrin IX
• symptoms include:
- skin rashes and blisters early in childhood
- cholestatic liver cirrhosis and progressive liver failure
- a chronic porphyria
- liver and erythroid tissues
- deficiency in uroporphyrinogen decarboxylase
- often no symptoms until 4th or 5th decade
lead poisoning
-inhibition of ferrochelatase ALA dehydratase
- displaces Zn+2 at enzyme active site
children
- developmental defects
- drop in IQ
- hyperactivity
- insomnia
- many other health problems
adults
- severe abdominal pain
- mental confusion
- many other symptoms