Invitrogen™ HMBS Polyclonal Antibody

Rabbit Polyclonal Antibody

Supplier:  Invitrogen™ PA597830

Catalog No. PIPA597830

Description

HMBS Polyclonal Antibody for Western Blot, ICC/IF, IHC (P), ELISA

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: Protein G
• Regulatory Status: RUO
• Gene ID (Entrez): 3145
• Content And Storage: -20°C or -80°C if preferred
• Form: Liquid

• Applications: ELISA, Immunohistochemistry (Paraffin), Western Blot, Immunocytochemistry
• Concentration: 5 mg/mL
• Formulation: PBS with 50% glycerol and 0.03% ProClin 300; pH 7.4
• Gene Accession No.: P08397
• Gene Symbols: Hmbs
• Immunogen: Recombinant Human Porphobilinogen deaminase protein (283-330AA).
• Quantity: 100 μg
• Primary or Secondary: Primary
• Target Species: Human
• Product Type: Antibody
• Isotype: IgG