Porphobilinogen deaminase

Porphobilinogen deaminase (PBG -D) (also Hydroxymethylbilan synthase HMBS ) is the enzyme that catalyzes the polymerization, followed by elimination reaction of four molecules of porphobilinogen catalyzes in all living beings Hydroxymethylbilan. PBG -D is therefore necessary for the porphyrin biosynthesis. Mutations in the HMBS gene of the people with the following lack of PBG -D can lead to acute intermittent porphyria.

PBG -D, there are two isoforms in humans, a red blood cell only encountered, and the other expressed in the other tissues.

Catalyzed reaction

4 H2O ⇒ 4 NH3

Four porphobilinogen molecules polymerize to form a molecule Hydroxymethylbilan, water being consumed and ammonia is produced. Dipyrromethane as a cofactor is necessary, which is bound and to which the individual porphobilinogen molecules are first added gradually.

Protein isoform Human Genome Organisation Transferase Organism Cofactor (biochemistry)
395169
de