Invitrogen™ HMBS Polyclonal Antibody

Catalog No.	Quantity
PIPA597830	100 μg

Catalog No. PIPA597830 Supplier Invitrogen™ Supplier No. PA597830

Description

Rabbit Polyclonal Antibody

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
• Applications: ELISA, Immunohistochemistry (Paraffin), Western Blot, Immunocytochemistry
• Classification: Polyclonal
• Concentration: 5 mg/mL
• Conjugate: Unconjugated
• Formulation: PBS with 50% glycerol and 0.03% ProClin 300; pH 7.4
• Gene: Hmbs
• Gene Accession No.: P08397
• 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
• Gene Symbols: Hmbs
• Host Species: Rabbit
• Immunogen: Recombinant Human Porphobilinogen deaminase protein (283-330AA).
• Purification Method: Protein G
• Quantity: 100 μg
• Regulatory Status: RUO
• Primary or Secondary: Primary
• Gene ID (Entrez): 3145
• Target Species: Human
• Content And Storage: -20°C or -80°C if preferred
• Product Type: Antibody
• Form: Liquid
• Isotype: IgG