HMBS antibody (Biotin)

Catalog No. ABIN1400653

Product Details anti-HMBS Antibody (Biotin)

Target: HMBS (Hydroxymethylbilane Synthase (HMBS))

Reactivity: Human, Mouse, Rat

Host: Rabbit

Clonality: Polyclonal

Conjugate: This HMBS antibody is conjugated to Biotin

Application: Western Blotting (WB), Immunohistochemistry (Paraffin-embedded Sections) (IHC (p))

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: WB 1:300-5000
IHC-P 1:200-400

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 for 12 months.

Expiry Date: 12 months

Target Details for HMBS

Target: HMBS (Hydroxymethylbilane Synthase (HMBS))

Alternative Name: HMBS (HMBS Products)

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