HMBS protein (His tag) (80R-2177)
Purified recombinant Human HMBS protein (His tag)
|Synonyms||UPS protein, PBG-D protein, PBG-D protein, Porphobilinogen deaminase protein, UPS protein, PBGD protein, PORC protein, Hydroxymethylbilane Synthase protein, PBGD protein|
Coomassie Blue stained SDS-PAGE of HMBS protein (His tag) (80R-2177)
Figure annotation denotes ug of protein loaded and % gel used.
|Residues||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|
|Purity||> 95% pure|
|Molecular Weight||41.9 kDa|
|Form & Buffer||Supplied as a liquid in 20mM Tris-HCl buffer (pH 8.0) containing 1mM DTT, 10% glycerol, 0.1M NaCl|
Storage & Safety
|Storage||Store at 4 deg C for short term storage. Aliquot and store at -20 deg C or - 70 deg C for long term storage. Avoid repeated freeze/thaw cycles.|
|Biological Significance||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.|
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