HMBS Polyclonal Antibody, Bethyl Laboratories

Rabbit Polyclonal Antibody

Supplier:  Bethyl Laboratories, Inc A304234AT

Catalog No. 50-156-7201

Description

The recommended shelf life for this product is 1 year from date of receipt.

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
• Formulation: TBS with 0.1% BSA and 0.09% sodium azide
• Gene Accession No.: P08397
• Gene Symbols: HMBS
• Immunogen: Between 311 and 361
• Quantity: 20 μL
• Primary or Secondary: Primary
• Target Species: Human
• Product Type: Antibody
• Isotype: IgG

• Applications: Western Blot
• Conjugate: Unconjugated
• Gene: HMBS
• Gene Alias: HEM3, HMBS, Hydroxymethylbilane synthase, PBG D, PBG-D, PBGD, Porphobilinogen deaminase, Pre uroporphyrinogen synthase, Pre-uroporphyrinogen synthase, UPS, Uroporphyrinogen I synthase, Uroporphyrinogen I synthetase
• Host Species: Rabbit
• Purification Method: Antigen affinity chromatography
• Regulatory Status: RUO
• Gene ID (Entrez): 3145
• Content And Storage: 4° C
• Form: Liquid