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PBGD Protein Crystal

(CBCRY24)  Download Datasheet
Background
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 Classification
Transferase
Structure Weight
80152.23 Da
Polymer
1
Molecule
Porphobilinogen deaminase
Chain Length
364 amino acids
Crystal Description
PDB ID
3ECR
MMDB ID
67075
Source
E.coli
Method
X-Ray Diffraction
Resolution
2.18Å
Ligand Chemical Component
DPM
Gene Information
Gene Name
HMBS
Synonyms
PBG-D; PBGD; UPS; porphobilinogen deaminase; pre-uroporphyrinogen synthase; uroporphyrinogen I synthase; uroporphyrinogen I synthetase; EC 2.5.1.61; hydroxymethylbilane synthase
UniProt ID
P08397
Gene ID
3145
Chromosome Location
11q23.3
Function
hydroxymethylbilane synthase activity; transferase activity
Reference
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