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    Which enzyme deficiency is present in congenital erythropoietic porphyria?

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    Which enzyme deficiency is present in congenital erythropoietic porphyria?

    a) UPG III co-synthase
    b) ALA synthase
    c) ferrochelatase
    d) UPG I synthase
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    Quote Originally Posted by maixungphong
    Which enzyme deficiency is present in congenital erythropoietic porphyria?
    a) UPG III co-synthase
    b) ALA synthase
    c) ferrochelatase
    d) UPG I synthase
    I believe that it's A) UPG III co-synthase... but am not 100% sure; need to look it up and get back to you...
    "SLAM-DUNK THE STEPS"

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    Hope that this helps....

    Congenital erythropoietic porphyria (step 4). Congenital erythropoietic porphyria (CEP) is also called Gunther's disease, erythropoietic porphyria, congenital porphyria, congenital hematoporphyria, and erythropoietic uroporphyria. CEP is inherited in an autosomal recessive manner. It is a rare disease, estimated to affect fewer than one in one million people. Onset of dramatic symptoms usually occurs in infancy, but may hold off until adulthood.

    The heme "assembly line" is an eight-step process, requiring eight different and properly functioning enzymes:

    • delta-aminolevulinic acid synthase
    • delta-aminolevulinic acid dehydratase
    • porphobilogen deaminase
    • uroporphyrinogen III cosynthase
    • uroporphyrinogen decarboxylase
    • coproporphyrinogen oxidase
    • protoporphyrinogen oxidase
    • ferrochelatase
    http://www.healthatoz.com/healthatoz...porphyrias.jsp (source)
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    maixungphong's Avatar
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    Correct! Correct!Correct!
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