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|Product Name||HMBS, 1-361aa, Human|
|Amino Acid Sequence||MGSSHHHHHH 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|
|Background||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. Recombinant human HMBS protein, fused to His-tag at N-terminus, was expressed in E.coli and purified by using conventional chromatography techniques.|
|Concentration||1 mg/ml (determined by Bradford assay)|
|Form||Liquid, in 20mM Tris-HCl buffer (pH8.0) containing 20% glycerol, 1mM DTT|
|Intended Use||For Research Use Only|
|Molecular Weight||40.3 kDa (380aa) confirmed by MALDI-TOF (Molecular size on SDS-PAGE will appear higher)|
|NCBI Accession #||NP_000181|
|Purity||> 95% by SDS-PAGE|
|References||Schneider Yin X., et al. (2004) J Inherit Metab Dis. 625-631:471-474.
Sheppard L., et al. (1995) Paediatr Anaesth. 15:426-428.
|Storage||Can be stored at +4C short term (1-2 weeks). For long term storage, aliquot and store at -20C or -70C. Avoid repeated freezing and thawing cycles.|
|Synonyms||Porphobilinogen deaminase, PBG-D, PBGD, PORC, UPS|
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