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Porphobilinogen

Intermediate in the biosynthesis of porphyrins

Intermediate in the biosynthesis of porphyrins

Porphobilinogen (PBG) is an organic compound that occurs in living organisms as an intermediate in the biosynthesis of porphyrins, which include critical substances like hemoglobin and chlorophyll.

The structure of the molecule can be described as molecule of pyrrole with sidechains substituted for hydrogen atoms at positions 2, 3 and 4 in the ring (1 being the nitrogen atom); respectively, an aminomethyl group , an acetic acid (carboxymethyl) group , and a propionic acid (carboxyethyl) group .

Biosynthesis

In a early step of the porphyrin biosynthesis pathway, porphobilinogen is generated from aminolevulinic acid (ALA) by the enzyme ALA dehydratase.

Metabolism

Next in the pathway towards porphyrins, four molecules of porphobilinogen are combined into hydroxymethyl bilane by the enzyme porphobilinogen deaminase:

Pathologies

Acute intermittent porphyria causes an increase in urinary porphobilinogen.

References

References

  1. {{cite encyclopedia. Paul R. Ortiz de Montellano. (2008). John Wiley & Sons
  2. (2000). "Tetrapyrroles: The pigments of life". Natural Product Reports.
  3. Voet, Donald. (2011). "Biochemistry". Wiley.
  4. {{KEGG enzyme. 2.5.1.61
  5. Aarsand, AK. (April 2006). "Estimation and application of biological variation of urinary delta-aminolevulinic acid and porphobilinogen in healthy individuals and in patients with acute intermittent porphyria". Clinical Chemistry.
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metabolism pyrroles carboxylic-acids aminomethyl-compounds
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