HMBS anticorps (Middle Region)

N° du produit ABIN2785719

Cité dans 1 publication. Cet anticorps anti-HMBS Polyclonal Lapin (ABIN2785719) détecte spécifiquement HMBS dans WB. L’anticorps est réactif avec des échantillons de Humain, Souris, Rat, Chien, Cheval, Lapin, Boeuf (Vache), Cobaye, Mouton, Poisson zèbre (Danio rerio) et Chévre.

Aperçu rapide pour HMBS anticorps (Middle Region) (ABIN2785719)

Antigène: HMBS (Hydroxymethylbilane Synthase (HMBS))

Reactivité: Humain, Souris, Rat, Chien, Cheval, Lapin, Boeuf (Vache), Cobaye, Mouton, Poisson zèbre (Danio rerio), Chévre

Hôte: Lapin

Clonalité: Polyclonal

Conjugué: Cet anticorp HMBS est non-conjugé

Application: Western Blotting (WB)

Détail du produit anti-HMBS anticorps

Épitope: Middle Region

Fonction: HMBS Antibody - middle region

Séquence: SSLRRAAQLQ RKFPHLEFRS IRGNLNTRLR KLDEQQEFSA IILATAGLQR

Homologie: Cow: 93%, Dog: 93%, Goat: 93%, Guinea Pig: 100%, Horse: 93%, Human: 100%, Mouse: 93%, Rabbit: 100%, Rat: 100%, Sheep: 93%, Zebrafish: 91%

Attributs du produit: This is a rabbit polyclonal antibody against HMBS. It was validated on Western Blot using a cell lysate as a positive control.

Purification: Affinity Purified

Immunogène: The immunogen is a synthetic peptide directed towards the middle region of human HMBS

Information d'application

Indications d'application: Optimal working dilution should be determined by the investigator.

Commentaires:

We also sell a specific blocking peptide that can be used in combination with this antibody. You can find the blocking peptide under ABIN8102864

Restrictions: For Research Use only

Stockage

Format: Liquid

Concentration: 0.5 mg/mL

Buffer: Purified antibody supplied in 1x PBS buffer with 0.09 % (w/v) sodium azide and 2 % sucrose.

Agent conservateur: Sodium azide

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

Conseil sur la manipulation: prevent freeze-thaw cycles

Stock: 4 °C,-20 °C

Stockage commentaire: For short term use, store at 2-8C up to 1 week. For long term storage, store at -20 °C in small aliquots to prevent freeze-thaw cycles.

Références pour anticorps anti-HMBS (ABIN2785719)

Kuo, Lee, Chuang, Huang: "Acute intermittent porphyria with peripheral neuropathy: a follow-up study after hematin treatment." dans: Journal of the neurological sciences, Vol. 260, Issue 1-2, pp. 231-5, (2007) (PubMed).

Détails sur HMBS

Antigène: HMBS (Hydroxymethylbilane Synthase (HMBS))

Autre désignation: HMBS

Sujet:

Background Information: HMBS is a member of the hydroxymethylbilane synthase superfamily. It is the third enzyme of the heme biosynthetic pathway and catalyzes the head to tail condensation of four porphobilinogen molecules into the linear hydroxymethylbilane. Mutations in this gene are associated with the autosomal dominant disease acute intermittent porphyria.This gene encodes a member of the hydroxymethylbilane synthase superfamily. The encoded protein is the third enzyme of the heme biosynthetic pathway and catalyzes the head to tail condensation of four porphobilinogen molecules into the linear hydroxymethylbilane. Mutations in this gene are associated with the autosomal dominant disease acute intermittent porphyria. Alternatively spliced transcript variants encoding different isoforms have been described.

Gene Name: Hydroxymethylbilane synthase

Alternative Symbols: UPS, PBGD, PORC, PBG-D

Protein Name: Porphobilinogen deaminase

Poids moléculaire: 40kDa

ID gène: 3145

NCBI Accession: NP_000181

UniProt: P08397