Porphobilinogen

Porphobilinogen (pronunciation: /ˌpɔːrfəʊˌbɪlɪˈnɒdʒɪn/) is a pyrrole-containing compound that is part of the heme biosynthetic pathway. It is an intermediate in the synthesis of porphyrin, which is a component of heme, the prosthetic group of hemoglobin.

Etymology

The term "Porphobilinogen" is derived from the Greek words "porphyra", meaning purple, and "bilin", referring to bile. The "-gen" suffix indicates that it is a precursor in the production of other compounds.

Biochemical Role

Porphobilinogen is produced in the cytoplasm of cells during the synthesis of heme. It is formed from the condensation of two molecules of aminolevulinic acid (ALA) in a reaction catalyzed by the enzyme ALA dehydratase. The resulting porphobilinogen is then converted into hydroxymethylbilane by the enzyme porphobilinogen deaminase.

Clinical Significance

Abnormal levels of porphobilinogen in the body can lead to a group of disorders known as porphyrias. These disorders are characterized by the accumulation of porphyrins or porphyrin precursors, such as porphobilinogen, due to defects in the heme biosynthetic pathway.

Related Terms

External links

Medical encyclopedia article on Porphobilinogen
Wikipedia's article - Porphobilinogen

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Porphobilinogen