HMBS, Recombinant, Human, aa1-361, His-Tag (Porphobilinogen Deaminase, PBG-D, Hydroxymethylbilane Synthase, HMBS, Pre-uroporphyrinogen Synthase, PBGD, UPS)

Supplier:

United States Biological

Cat no: 045433

HMBS, Recombinant, Human, aa1-361, His-Tag (Porphobilinogen Deaminase, PBG-D, Hydroxymethylbilane Synthase, HMBS, Pre-uroporphyrinogen Synthase, PBGD, UPS)

Porphobilinogen deaminase, also known as HMBS, is a member of the hydroxymethylbilane synthase superfamily. It is a cytoplasmic enzyme found in the heme synthesis pathway. Deficiency of HMBS causes errors in pyrrole metabolism which in turn leads to an inherited autosomal disorder called acute intermittent porphyria (AIP) which is characterized by acute attacks of neurological dysfunctions with hypertension, tachycardia, peripheral neurologic disturbances, abdominal pain and excessive amounts of aminolevulinic acid and porphobilinogen in the urine.\n\nSource:\nRecombinant corresponding to aa1-361 from human HMBS, fused to His-tag at N-terminus, expressed in E.coli.\n\nMolecular Weight:\n~41.9kD (385aa) confirmed by MALDI-TOF\n\nAA Sequence:\nMGSSHHHHHH SSGLVPRGSH MGSHMSGNGN AAATAEENSP KMRVIRVGTR KSQLARIQTD SVVATLKASY PGLQFEIIAM STTGDKILDT ALSKIGEKSL FTKELEHALE KNEVDLVVHS LKDLPTVLPP GFTIGAICKR ENPHDAVVFH PKFVGKTLET LPEKSVVGTS SLRRAAQLQR KFPHLEFRSI RGNLNTRLRK LDEQQEFSAI ILATAGLQRM GWHNRVGQIL HPEECMYAVG QGALGVEVRA KDQDILDLVG VLHDPETLLR CIAERAFLRH LEGGCSVPVA VHTAMKDGQL YLTGGVWSLD GSDSIQETMQ ATIHVPAQHE DGPEDDPQLV GITARNIPRG PQLAAQNLGI SLANLLLSKG AKNILDVARQ LNDAH\n\nStorage and Stability:\nMay be stored at 4 degrees C for short-term only. Aliquot to avoid repeated freezing and thawing. Store at -20 degrees C. Aliquots are stable for 6 months at -20 degrees 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.

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SPECIFICATIONS

Catalog Number

045433

Size

100ug

Form

Supplied as a liquid in 20mM Tris-HCl, pH 8.0, 1mM DTT, 0.1M sodium chloride, 10% glycerol.

Purity

~95% (SDS-PAGE)

References

1. Schneider Yin X., et al. (2004) J Inherit Metab Dis. 625-631:471-474. 2. Sheppard L., et al. (1995) Paediatr Anaesth. 15:426-428.

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SUPPLIER INFO

United States Biological

Address:

4 Technology Way, Salem, MA 01970, US

Phone:

+1 800 520 3011

Website:

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