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8 Cards in this Set

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succinyl CoA + glycine ----> ALA
ALA synthase + Vitamin B6
-Committed step
-Negative feedback via heme
-In Mitochondria
ALA --->PBG
ALA dehydrogenase or PBG Synthase
Target for Pb poisoning
oxidations and decarboxylations to produce protoporphyrin IX
acetates to methyles
proprionates to vinyls
Protoporphyrin ---> Heme
Ferrochelatase
Mitochondria
Acute Intermittent Porphyria
Block Before formation of porphryinogen therefore ALA and PBG accumulate
ALA buildup inhibits ATPase affects nerve conduction
Congenital Erythopoietic Porphyria
block after formation of porphyrinogens
Porphyrinogens accumulate
Oxidation of Porphyrinogens cause photosensivity, skin damage
The effect of lead of ALA
Stimulates ALA synthetase by inhibiting ALA dehydrogense

Patients have high ALA
Affect of lead on Fe
Low ferrochelatase (which inserts Fe+2 into protoporphyrin ring)---> high Protoporphyrin IX

In blood, see Zn protoporhyrin because Zn inserts instead of Fe