HMBS anticorps (N-Term)

N° du produit ABIN2785718

Détail du produit anti-HMBS anticorps

Antigène: HMBS (Hydroxymethylbilane Synthase (HMBS))

Épitope: N-Term

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

Hôte: Lapin

Clonalité: Polyclonal

Conjugué: Cet anticorp HMBS est non-conjugé

Application: Western Blotting (WB)

Séquence: MRVIRVGTRK SQLARIQTDS VVATLKASYP GLQFEIIAMS TTGDKILDTA

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

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 N terminal region of human HMBS

Information d'application

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

Commentaires:

Antigen size: 344 AA

Restrictions: For Research Use only

Stockage

Format: Liquid

Concentration: Lot specific

Buffer: Liquid. 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: Avoid repeated freeze-thaw cycles.

Stock: -20 °C

Stockage commentaire: For short term use, store at 2-8°C 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 (ABIN2785718)

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 (HMBS Produits)

Sujet: 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.
Alias Symbols: PBG-D, PBGD, UPS, PORC
Protein Interaction Partner: RANBP9, APP, UBC,
Protein Size: 344

Poids moléculaire: 38 kDa

ID gène: 3145

NCBI Accession: NM_001024382, NP_001019553

UniProt: P08397