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porphyrin basics
biosynthesis of porphyrin
types of porphyrias
regulation
enzyme defects
oral box
porphyrin basics
cyclic compounds formed by fusion of 4 pyrrole rings linked by methenyl bridges
biosynthesis of porphyrin
synthesised by almost all tissues
main organ - liver, bone marrow
heme synthesised in normoblasts but not in matured erythrocytes
pathway is partly cytoplasmic + mitochondrial
types of porphyrias
porphyria
rare diseases due to abnormalities during synthesis of heme
can be genetic or acquired
regulation
enzyme defects
oral box