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PBGD Protein Crystal(CBCRY24)

Product Summary
Name PBGD Protein Crystal Download Datasheet
Background The protein is the third enzyme of the heme biosynthetic pathway and catalyzes the head to tail condensation of four porphobilinogen molecules into the linear hydroxymethylbilane. Mutations in this gene are associated with the autosomal dominant disease acute intermittent porphyria. Alternatively spliced transcript variants encoding different isoforms have been described.
Molecular Description
Protein ClassificationTransferase
Structure Weight80152.23 Da
MoleculePorphobilinogen deaminase
Chain Length364 amino acids
Crystal Description
MMDB ID67075
MethodX-Ray Diffraction
Ligand Chemical ComponentDPM
Gene Information
Gene NameHMBS
SynonymsPBG-D; PBGD; UPS; porphobilinogen deaminase; pre-uroporphyrinogen synthase; uroporphyrinogen I synthase; uroporphyrinogen I synthetase; EC; hydroxymethylbilane synthase
UniProt IDP08397
Gene ID3145
Chromosome Location11q23.3
Functionhydroxymethylbilane synthase activity; transferase activity
Reference Song, G., Li, Y., Cheng, C., Zhao, Y., Gao, A., Zhang, R., Joachimiak, A., Shaw, N., Liu, Z.J. (2009) Structural insight into acute intermittent porphyria. Faseb J. 23: 396-404

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