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Product Name | HMBS, Recombinant, Human, aa1-361, His-Tag |
Description | Purity ~95% (SDS-PAGE). 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. Source: Recombinant corresponding to aa1-361 from human HMBS, fused to His-tag at N-terminus, expressed in E.coli. Molecular Weight: ~41.9kD (385aa) confirmed by MALDI-TOF AA Sequence: MGSSHHHHHH SSGLVPRGSH MGSHMSGNGN AAATAEENSP KMRVIRVGTR KSQLARIQTD SVVATLKASY PGLQFEIIAM STTGDKILDT ALSKIGEKSL FTKELEHALE KNEVDLVVHS LKDLPTVLPP GFTIGAICKR ENPHDAVVFH PKFVGKTLET LPEKSVVGTS SLRRAAQLQR KFPHLEFRSI RGNLNTRLRK LDEQQEFSAI ILATA |
Size | 100ug |
Concentration | n/a |
Applications | n/a |
Other Names | HMBS, Recombinant, Human, aa1-361, His-Tag (Porphobilinogen Deaminase, PBG-D, Hydroxymethylbilane Synthase, HMBS, Pre-uroporphyrinogen Synthase, PBGD, UPS) |
Gene, Accession, CAS # | n/a |
Catalog # | 45433 |
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Order / More Info | HMBS, Recombinant, Human, aa1-361, His-Tag from UNITED STATES BIOLOGICAL |
Product Specific References | n/a |
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