Invitrogen™ HMBS Polyclonal Antibody

Rabbit Polyclonal Antibody

Brand:  Invitrogen™ PA562230

Product Code. 15790022

Description

Immunogen sequence: EFSAIILATA GLQRMGWHNR VGQILHPEEC MYAVGQGALG VEVRAKDQDI LDLVGVLHDP ETLLRCIAER AFLRHLEGGC SVPVAVHTAM KDGQLYLTGG VWSLDGSDSI QETMQATIHV PAQHEDGPED DPQLV Highest antigen sequence identity to the following orthologs: Mouse - 93%, Rat - 94%.

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

• Antigen: HMBS
• Classification: Polyclonal
• Conjugate: Unconjugated
• Gene: Hmbs
• 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
• Host Species: Rabbit
• Purification Method: Antigen affinity chromatography
• Regulatory Status: RUO
• Gene ID (Entrez): 3145
• Content And Storage: Store at 4°C short term. For long term storage, store at -20°C, avoiding freeze/thaw cycles.
• Form: Liquid

• Applications: Immunohistochemistry (Paraffin), Western Blot, Immunocytochemistry
• Concentration: 0.4 mg/mL
• Formulation: PBS with 40% glycerol and 0.02% sodium azide; pH 7.2
• Gene Accession No.: P08397
• Gene Symbols: Hmbs
• Immunogen: Recombinant protein corresponding to Human HMBS. Recombinant protein control fragment (Product #RP-89537).
• Quantity: 100 μL
• Primary or Secondary: Primary
• Target Species: Human
• Product Type: Antibody
• Isotype: IgG