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Porphobilinogen synthase

 
Wikipedia: Porphobilinogen synthase
Porphobilinogen synthase
Identifiers
Symbol ALAD
Entrez 210
HUGO 395
OMIM 125270
RefSeq NM_001003945
UniProt P13716
Other data
EC number 4.2.1.24
Locus Chr. 9 q32

Porphobilinogen synthase (or ALA dehydratase, or Aminolevulinate dehydratase) synthesizes porphobilinogen through the asymmetric condensation of two molecules of aminolevulinic acid. All natural tetrapyrroles, including hemes, chlorophylls and vitamin B12, share porphobilinogen as a common precursor.

It is involved in the second step of the metabolism of porphyrin.

Clinical significance

Porphobilinogen synthase deficiency is a rare cause of porphyria.[1]


Heme synthesis—note that some reactions occur in the cytoplasm and some in the mitochondrion (yellow)

Lead poisoning works on the cellular level by binding to this enzyme, rendering it useless.

References

  1. ^ Jaffe EK, Stith L (February 2007). "ALAD porphyria is a conformational disease". Am. J. Hum. Genet. 80 (2): 329–37. doi:10.1086/511444. PMID 17236137. 

External links


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Wikipedia. This article is licensed under the Creative Commons Attribution/Share-Alike License. It uses material from the Wikipedia article "Porphobilinogen synthase" Read more