ALAD Enzyme: The Critical Second Step in Heme Synthesis and its Link to Porphyrias

reaction 2 of heme synthesis

location: cytosolenzyme: porphobilinogen synthaseporphobilinogen synthesis. 2 molecules of ALA required for each porphobilinogen molecule. 8 molecules of ALA are needed to make 4 porphobilinogen for the next step

The second reaction of heme synthesis is carried out by the enzyme Delta-Aminolevulinic Acid Dehydratase (ALAD). This reaction involves the conversion of delta-aminolevulinic acid (ALA) to porphobilinogen (PBG), which is a cyclic compound consisting of four pyrrole rings.

The reaction is a condensation reaction where two molecules of ALA form a Schiff base intermediate, which is then cyclized to form PBG. The reaction requires the presence of a cofactor called zinc and also proceeds with the consumption of one molecule of water to produce one molecule of PBG.

The reaction occurs in the cytoplasm of the cell and represents a critical step in the biosynthesis of heme. Abnormalities in the activity of ALAD have been linked to several diseases, including porphyrias, which are a group of inherited disorders resulting from abnormalities in heme synthesis.

More Answers:

Understanding the Crucial Role of Reactions 5 and 6 in Heme Synthesis: From PBG to HMB
Understanding the Essential Role of Uroporphyrinogen Decarboxylase in Heme Synthesis and its Link to Porphyria
Understanding Heme Biosynthesis: The Significance of Uroporphyrinogen III Formation in the Cytoplasm

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