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    Heme Synthesis

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    jdf
    The document summarizes the heme synthesis pathway which occurs both in the mitochondria and cytosol of cells. Key enzymes in the pathway convert glycine and succinyl-CoA into delta-aminolevulinic acid (ALA) in the mitochondria. ALA is then converted through multiple steps involving other enzymes into protoporphyrinogen IX and finally heme with the insertion of iron by ferrochelatase.

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    Heme Synthesis

    Uploaded by

    jdf
    5 views1 page
    The document summarizes the heme synthesis pathway which occurs both in the mitochondria and cytosol of cells. Key enzymes in the pathway convert glycine and succinyl-CoA into delta-aminolevulinic acid (ALA) in the mitochondria. ALA is then converted through multiple steps involving other enzymes into protoporphyrinogen IX and finally heme with the insertion of iron by ferrochelatase.

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    The document summarizes the heme synthesis pathway which occurs both in the mitochondria and cytosol of cells. Key enzymes in the pathway convert glycine and succinyl-CoA into delta-aminolevulinic acid (ALA) in the mitochondria. ALA is then converted through multiple steps involving other enzymes into protoporphyrinogen IX and finally heme with the insertion of iron by ferrochelatase.

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    © All Rights Reserved
    Download as PDF, TXT or read online from Scribd
    Flag for inappropriate content
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    5 views1 page

    Heme Synthesis

    Uploaded by

    jdf
    The document summarizes the heme synthesis pathway which occurs both in the mitochondria and cytosol of cells. Key enzymes in the pathway convert glycine and succinyl-CoA into delta-aminolevulinic acid (ALA) in the mitochondria. ALA is then converted through multiple steps involving other enzymes into protoporphyrinogen IX and finally heme with the insertion of iron by ferrochelatase.

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    HEME SYNTHESIS

    Glycine Succinyl-CoA

    ALA synthase
    CO2, CoA
    PLP

    δ- aminolevulinic acid (ALA)


    MITOCHONDRIA

    δ- aminolevulinic acid (ALA)


    ALA
    ALA dehydratase
    2H2O
    Porphobilinogen (PBG)
    PBG deaminase/ 3 PBG
    hydroxymethylbilane synthase/
    uroporphyrinogen I synthase
    4 NH3
    Hydroxymethylbilane
    CYTOSOL
    Uroporphyrinogen III
    cosynthase

    Uroporphyrinogen I Uroporphyrinogen III


    Uroporphyrinogen
    4 CO2 decarboxylase
    4 CO2
    Coproporphyrinogen I Coproporphyrinogen III

    MITOCHONDRIA
    Coproporphyrinogen III
    O2
    Coproporphyrinogen
    oxidase
    2 CO2
    Protoporphyrinogen IX

    Protoproporphyrinogen
    oxidase 4 H+

    Protoporphyrin IX

    Fe++
    Ferrochelatase
    2 H+
    Heme

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