Recombinant Human HMBS protein, His-tagged

• Cat.No.: HMBS-39H
• Product Overview: Recombinant Human HMBS(Ser2-His361) fused with His tag at C-terminal was expressed in HEK293.

Specification

• Description: 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.
• Source: HEK293
• Species: Human
• Tag: His
• Form: Lyophilized from a 0.2 μm filtered solution of 20mM PB,150mM NaCl,pH7.4.
• AA Sequence: SGNGNAAATAEENSPKMRVIRVGTR KSQLARIQTDSVVATLKASYPGLQF EIIAMSTTGDKILDT ALSKIGEKSLFTKELEHALEKNEVD LVVHSLKDLPTVLPPGFTIGAICKR ENPHDAVVFHPKFVG KTLETLPEKSVVGTSSLRRAAQLQR KFPHLEFRSIRGNLNTRLRKLDEQQ EFSAIILATAGLQRM GWHNRVGQILHPEECMYAVGQGALG VEVRAKDQDILDLVGVLHDPETLLR CIAERAFLRHLEGGC SVPVAVHTAMKDGQLYLTGGVWSLD GSDSIQETMQATIHVPAQHEDGPED DPQLVGITARNIPRG PQLAAQNLGISLANLLLSKGAKNIL DVARQLNDAHVDHHHHHH
• Endotoxin: Less than 0.1 ng/µg (1 IEU/µg) as determined by LAL test.
• Purity: Greater than 95% as determined by reducing SDS-PAGE.
• Storage: Lyophilized protein should be stored at < -20 centigrade, though stable at room temperature for 3 weeks.Reconstituted protein solution can be stored at 4-7 centigrade for 2-7 days.Aliquots of reconstituted samples are stable at < -20 centigrade for 3 months.
• Reconstitution: Always centrifuge tubes before opening. Do not mix by vortex or pipetting.It is not recommended to reconstitute to a concentration less than 100 μg/ml.Dissolve the lyophilized protein in ddH2O.Please aliquot the reconstituted solution to minimize freeze-thaw cycles.

Gene Information

• Gene Name: HMBS hydroxymethylbilane synthase [ Homo sapiens ]
• Official Symbol: HMBS
• Synonyms: HMBS; hydroxymethylbilane synthase; PBGD, PORC, porphobilinogen deaminase , porphyria, acute; Chester type , UPS, uroporphyrinogen I synthase; porphobilinogen deaminase; uroporphyrinogen I synthase; pre-uroporphyrinogen synthase; uroporphyrinogen I synthetase; porphyria, acute; Chester type; UPS; PBGD; PORC; PBG-D
• Gene ID: 3145
• mRNA Refseq: NM_000190
• Protein Refseq: NP_000181
• MIM: 609806
• UniProt ID: P08397
• Chromosome Location: 11q23.2-qter
• Pathway: Heme Biosynthesis, organism-specific biosystem; Heme biosynthesis, organism-specific biosystem; Metabolic pathways, organism-specific biosystem; Metabolism, organism-specific biosystem; Metabolism of porphyrins, organism-specific biosystem; Porphyrin and chlorophyll metabolism, organism-specific biosystem; Porphyrin and chlorophyll metabolism, conserved biosystem
• Function: amine binding; carboxylic acid binding; coenzyme binding; hydroxymethylbilane synthase activity; hydroxymethylbilane synthase activity; transferase activity; uroporphyrinogen-III synthase activity