HMBS anticorps (AbBy Fluor® 350)

N° du produit ABIN1400649

Cet anticorps anti-HMBS est un anticorps Lapin Polyclonal détectant HMBS dans WB et IF (p). Adapté pour Humain, Souris et Rat.

Aperçu rapide pour HMBS anticorps (AbBy Fluor® 350) (ABIN1400649)

Antigène: HMBS (Hydroxymethylbilane Synthase (HMBS))

Reactivité: Humain, Souris, Rat

Hôte: Lapin

Clonalité: Polyclonal

Conjugué: Cet anticorp HMBS est conjugé à/à la AbBy Fluor® 350

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

Détail du produit anti-HMBS anticorps (AbBy Fluor® 350)

Réactivité croisée: Humain, Souris, Rat

Purification: Purified by Protein A.

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

Isotype: IgG

Information d'application

Indications d'application: IF(IHC-P) 1:50-200

Restrictions: For Research Use only

Stockage

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.

Agent conservateur: ProClin

Précaution d'utilisation: This product contains ProClin: a POISONOUS AND HAZARDOUS SUBSTANCE, which should be handled by trained staff only.

Stock: -20 °C

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

Date de péremption: 12 months

Détails sur HMBS

Antigène: HMBS (Hydroxymethylbilane Synthase (HMBS))

Autre désignation: HMBS

Sujet:

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.

ID gène: 3145