HMBS anticorps (Biotin)

N° du produit ABIN1400653

Détail du produit anti-HMBS anticorps (Biotin)

Antigène: HMBS (Hydroxymethylbilane Synthase (HMBS))

Reactivité: Humain, Souris, Rat

Hôte: Lapin

Clonalité: Polyclonal

Conjugué: Cet anticorp HMBS est conjugé à/à la Biotin

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

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

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 for 12 months.

Date de péremption: 12 months

Détails sur HMBS

Antigène: HMBS (Hydroxymethylbilane Synthase (HMBS))

Autre désignation: HMBS (HMBS Produits)

Synonymes: anticorps PBG-D, anticorps PBGD, anticorps PORC, anticorps UPS, anticorps URO-S, anticorps hemC, anticorps pbg-d, anticorps pbgd, anticorps ups, anticorps SPAC806.01, anticorps DDBDRAFT_0186148, anticorps DDBDRAFT_0231417, anticorps DDB_0186148, anticorps DDB_0231417, anticorps Hmbs, anticorps NV50236, anticorps T25658, anticorps Ups, anticorps Uros1, anticorps hmbs, anticorps zgc:64128, anticorps hmbsl, anticorps id:ibd5004, anticorps im:7140060, anticorps zgc:110690, anticorps hydroxymethylbilane synthase, anticorps hydroxymethylbilane synthase S homeolog, anticorps hydroxymethylbilane synthase L homeolog, anticorps hydroxymethylbilane synthase (predicted), anticorps Hydroxymethylbilane synthase, anticorps hydroxymethylbilane synthase a, anticorps hydroxymethylbilane synthase, b, anticorps HMBS, anticorps Hmbs, anticorps hmbs, anticorps hmbs.2.S, anticorps hmbs.L, anticorps CNC02250, anticorps hem3, anticorps hemC, anticorps Trad_0335, anticorps Plabr_2100, anticorps Sgly_3113, anticorps SMLT_RS19660, anticorps PMI_RS16580, anticorps hmbsa, anticorps hmbsb

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