MS9 Porphyrias

- Porphyrin synthesis:
- A) Pyrrole ring
- B) Porphin ring
- C) protoporphyrin IX
- Heme = protoporphyrin IX + Fe
- Clorophyll is the same structure + Mg
Heme biosynthesis
- Heme is made in every cell
- 85% is made in erythroid cells
- There are 8 enzymatic reactions in the process
- Mutations in the enzymes that control these reactions lead to the disorders known as porphyria
- Porphyrias
- -Green: Hepatic porphyrias
- -Red: Erythropoietic porphyrias
- δALA - δ-aminolevulinic acid
- First step of heme synthesis: Rate limiting
- Two forms of ALA Synthase (ALAS)
- -ALAS2: erythroid-specific form
- -ALAS1: ubiquitous form responsible for the production of heme in the liver

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jknell

207529

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MS9 Porphyrias

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Porphyrias

Updated

2013-03-15T22:38:01Z

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