HMBS antibody (AbBy Fluor® 647)

Catalog No. ABIN1400652

This anti-HMBS antibody is a Rabbit Polyclonal antibody detecting HMBS in WB and IF (p). Suitable for Human, Mouse and Rat.

Quick Overview for HMBS antibody (AbBy Fluor® 647) (ABIN1400652)

Target: HMBS (Hydroxymethylbilane Synthase (HMBS))

Reactivity: Human, Mouse, Rat

Host: Rabbit

Clonality: Polyclonal

Conjugate: This HMBS antibody is conjugated to AbBy Fluor® 647

Application: Western Blotting (WB), Immunofluorescence (Paraffin-embedded Sections) (IF (p))

Product Details anti-HMBS Antibody (AbBy Fluor® 647)

Cross-Reactivity: Human, Mouse, Rat

Purification: Purified by Protein A.

Immunogen: KLH conjugated synthetic peptide derived from human HMBS/PBGD

Isotype: IgG

Application Details

Application Notes: IF(IHC-P) 1:50-200

Restrictions: For Research Use only

Handling

Format: Liquid

Concentration: 1 μg/μL

Buffer: Aqueous buffered solution containing 0.01M TBS ( pH 7.4) with 1 % BSA, 0.03 % Proclin300 and 50 % Glycerol.

Preservative: ProClin

Precaution of Use: This product contains ProClin: a POISONOUS AND HAZARDOUS SUBSTANCE, which should be handled by trained staff only.

Storage: -20 °C

Storage Comment: Store at -20°C. Aliquot into multiple vials to avoid repeated freeze-thaw cycles.

Expiry Date: 12 months

Target Details for HMBS

Target: HMBS (Hydroxymethylbilane Synthase (HMBS))

Alternative Name: HMBS

Background:

Synonyms: HEM3_HUMAN, HMBS, Hydroxymethylbilane synthase, PBG D, PBG-D, PBGD, Porphobilinogen deaminase, Pre uroporphyrinogen synthase, Pre-uroporphyrinogen synthase, UPS, Uroporphyrinogen I synthase, Uroporphyrinogen I synthetase.

Background: PBGD, 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.

Gene ID: 3145