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- Heme is a constituent of hemoglobin, myoglobin, and cytochromes - Heme is synthesized in most cells of the body- The liver is the main nonerythrocyte source of its synthesis- Heme is a porphyrin, a cyclic compound that contains four pyrrole rings linked together by methenyl bridges- It is synthesized from glycine and succinyl-coenzyme A, which condense to form 5-aminolevulinate (5-ALA)- This reaction is catalyzed by 5-ALA synthase, located in mitochondria, and is rate limiting in heme synthesis
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