Recombinant protein corresponding to human HMBS (Position: N6-H361), expressed in E. coli.
Porphobilinogen deaminase (hydroxymethylbilane synthase, or uroporphyrinogen I synthase) is an enzyme that in humans is encoded by the HMBS gene. This gene encodes a member of the hydroxymethylbilane synthase superfamily. The encoded 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. Applications: Suitable for use in ELISA, Western Blot. Other applications not tested. Recommended Dilution: ELISA: 0.1-0.5ug/ml Western Blot: 0.1-0.5ug/ml Optimal dilutions to be determined by the researcher. Storage and Stability: Lyophilized and reconstituted products are stable for 12 months after receipt at -20C. Reconstitute with sterile ddH2O. Aliquot to avoid repeated freezing and thawing. Store at -20C. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap. Further dilutions can be made in assay buffer.
