Invitrogen™ HMBS Polyclonal Antibody

Rabbit Polyclonal Antibody

Supplier:  Invitrogen™ PA537366

Catalog No. PIPA537366

Description

This antibody detects endogenous protein at a molecular weight of 39 kDa. Purity is >95% by SDS-PAGE.

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): 15288, 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: Western Blot
• Concentration: 1 mg/mL
• Formulation: PBS with 50% glycerol and 0.02% sodium azide; pH 7.2
• Gene Accession No.: P08397, P22907
• Gene Symbols: Hmbs
• Immunogen: Recombinant full length human HMBS.
• Quantity: 100 μL
• Primary or Secondary: Primary
• Target Species: Human, Mouse
• Product Type: Antibody
• Isotype: IgG

Safety and Handling

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