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/mlWestern Blot: 0.1-0.5ug/mlOptimal dilutions to be determined by the researcher.
Storage and Stability:Lyophilized powder may be stored at -20°C.
Stable for 12 months after receipt at -20°C.
Reconstitute with sterile ddH2O.
Aliquot to avoid repeated freezing and thawing.
Store at -20°C.
Reconstituted product is stable for 12 months at -20°C.
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.
仕様
Size：100ug
Host：rabbit
Source Antibody：human
Grade：Affinity Purified
Purity：Purified by immunoaffinity chromatography.
Form：Supplied as a lyophilized powder in 5mg/ml BSA, 0.9mg sodium chloride, 0.2mg Na2HPO4, 0.05% sodium azide. Reconstitute with 200ul sterile ddH2O to ~0.5mg/ml.
Specificity：Recognizes human HMBS. Species Crossreactivity: mouse, rat.
Isotype：IgG
Calc Applications Abbrev：E WB
Calc Crossreactivity：Hu Mo Rt
Immunogen：Recombinant protein corresponding to human HMBS (Position: N6-H361), expressed in E. coli.
