40 - Heme and hemoproteins. Biosynthesis of porphyrins – tissue and cellular localization, regulation. Types of porphyrias. Structure and types of hemoglobin.

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7 Terms

1
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section

  • porphyrin basics

  • biosynthesis of porphyrin

  • types of porphyrias

  • regulation

  • enzyme defects

  • oral box

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  • porphyrin basics

  • cyclic compounds formed by fusion of 4 pyrrole rings linked by methenyl bridges

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  • 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

<ul><li><p>synthesised by almost all tissues </p></li><li><p>main organ - liver, bone marrow </p></li><li><p>heme synthesised in normoblasts but not in matured erythrocytes </p></li><li><p>pathway is partly cytoplasmic + mitochondrial </p></li></ul><p></p>
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  • types of porphyrias

  • porphyria

    • rare diseases due to abnormalities during synthesis of heme

    • can be genetic or acquired

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  • regulation

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  • enzyme defects

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oral box

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