Porphobilinogen Deaminase/HMBS/PBGD, Recombinant Protein

Porphobilinogen Deaminase; PBG-D; Hydroxymethylbilane Synthase; HMBS; Pre-Uroporphyrinogen Synthase; HMBS; PBGD; UPS

For Research Use Only. Not for use in diagnostic procedures.

Human Cells

>95% as determined by reducing SDS-PAGE.

Lyophilized from a 0.2 mum filtered solution of 20mM PB, 150mM NaCl, pH 7.4.

Lyophilized protein should be stored at < -20 degree C, though stable at room temperature for 3 weeks.
Reconstituted protein solution can be stored at 4-7 degree C for 2-7 days. Aliquots of reconstituted samples are stable at < -20 degree C for 3 months.

Manufactured in an ISO 9001:2015 Certified Laboratory.

Small volumes of HMBS/PBGD recombinant protein vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.

Porphobilinogen Deaminase (HMBS) is a member of the HMBS family. PBGD is the third enzyme of the heme biosynthetic pathway and catalyzes the head to tail condensation of four porphobilinogen molecules into the linear hydroxymethylbilane. HMBS is involved in the production of heme, which is important for all of the body's organs, although it is most abundant in the blood, bone marrow, and liver. In addition, Heme is an essential component of iron-containing proteins called hemoproteins, including hemoglobin. Defects in PBGD are the cause of acute intermittent porphyria.

Molecular Mass: 40.5 kDa
Actual Protein Molecular Mass: 47 kDa

hydroxymethylbilane synthase

porphobilinogen deaminase

Porphobilinogen deaminase

PBGD; UPS; PBG-D; HMBS  [Similar Products]

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. [provided by RefSeq, Jul 2008]

Tetrapolymerization of the monopyrrole PBG into the hydroxymethylbilane pre-uroporphyrinogen in several discrete steps.

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