Invitrogen™ Human HMBS (aa 182-316) Control Fragment Recombinant Protein

Catalog No.	Quantity
RP89537	100 μL

Catalog No. RP89537 Supplier Invitrogen™ Supplier No. RP89537

Description

Recombinant Protein

Highest antigen sequence indentity to the following orthologs: Mouse (93%), Rat (93%). This recombinant protein control fragment may be used for blocking experiments with the corresponding antibody, PA5-62230. In IHC/ICC and WB experiments, we recommend a 100x molar excess of the protein fragment control based on the concentration and the molecular weight. Pre-incubate the antibody-protein control fragment mixture for 30 min at room temperature.

PBGD (porphobilinogen deaminase), also designated hydroxymethylbilane synthase, is a cytoplasmic enzyme found in the heme synthesis pathway. PBGD belongs to the HMBS (hydroxymethylbilane synthase) family. Deficiency of PBGD causes errors in pyrrole metabolism, which in turn leads to an inherited autosomal disorder called acute intermittent porphyria (AIP). AIP 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.

Specifications

• Accession Number: P08397
• Concentration: ≥5.0 mg/mL
• For Use With (Application): Neutralization, Control
• Formulation: PBS, 1M urea with no preservative; pH 7.4
• Gene ID (Entrez): 3145
• Name: Human HMBS (aa 182-316) Control Fragment
• pH Range: 7.4
• Purification Method: Purified
• Quantity: 100 μL
• Storage Requirements: -20°C, Avoid Freeze/Thaw Cycles
• Regulatory Status: RUO
• Gene Alias: alternative name: porphobilinogen deaminase; HEM3; hemC; HMBS; Hydroxymethylbilane synthase; PBG D; PBGD; PBG-D; PORC; Porphobilinogen deaminase; porphyria, acute; Chester type; pre-uroporphyrinogen synthase; similar to Porphobilinogen deaminase (Hydroxymethylbilane synthase) (HMBS) (Pre-uroporphyrinogen synthase) (PBG-D); T25658; Ups; uroporphyrinogen I synthase; uroporphyrinogen I synthetase; URO-S; Uros1
• Common Name: HMBS
• Gene Symbol: Hmbs
• Product Type: Protein
• Conjugate: Unconjugated
• Species: Human
• Recombinant: Recombinant
• Protein Tag: His-ABP-tag
• Sequence: EFSAIILATAGLQRMGWHNRVGQILHPEECMYAVGQGALGVEVRAKDQDILDLVGVLHDPETLLRCIAERAFLRHLEGGCSVPVAVHTAMKDGQLYLTGGVWSLDGSDSIQETMQATIHVPAQHEDGPEDDPQLV
• Content And Storage: -20°C, Avoid Freeze/Thaw Cycles
• Expression System: E. coli
• Form: Liquid
• Purity or Quality Grade: >80% by SDS-PAGE and Coomassie blue staining