COLQ anticorps (C-Term)

N° du produit ABIN1881222

Détail du produit anti-COLQ anticorps

Antigène: COLQ (AChE Q Subunit (COLQ))

Épitope: AA 324-353, C-Term

Reactivité: Humain, Souris

Hôte: Lapin

Clonalité: Polyclonal

Conjugué: Cet anticorp COLQ est non-conjugé

Application: Western Blotting (WB)

Homologie: Rat

Purification: This antibody is purified through a protein A column, followed by peptide affinity purification.

Immunogène: This COLQ antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide between 324-353 amino acids from the C-terminal region of human COLQ.

Clone: RB30589

Isotype: Ig Fraction

Information d'application

Indications d'application: WB: 1:1000

Restrictions: For Research Use only

Stockage

Format: Liquid

Buffer: Purified polyclonal antibody supplied in PBS with 0.09 % (W/V) sodium azide.

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.

Stock: 4 °C,-20 °C

Date de péremption: 6 months

Références pour anticorps anti-COLQ (ABIN1881222)

Mihaylova, Müller, Vilchez, Salih, Kabiraj, DAmico, Bertini, Wölfle, Schreiner, Kurlemann, Rasic, Siskova, Colomer, Herczegfalvi, Fabriciova, Weschke, Scola, Hoellen, Schara, Abicht, Lochmüller: "Clinical and molecular genetic findings in COLQ-mutant congenital myasthenic syndromes." dans: Brain : a journal of neurology, Vol. 131, Issue Pt 3, pp. 747-59, (2008) (PubMed).

Schreiner, Hoppenz, Klaeren, Reimann, Woelfle: "Novel COLQ mutation 950delC in synaptic congenital myasthenic syndrome and symptomatic heterozygous relatives." dans: Neuromuscular disorders : NMD, Vol. 17, Issue 3, pp. 262-5, (2007) (PubMed).

Ishigaki, Nicolle, Krejci, Leroy, Koenig, Fardeau, Eymard, Hantaï: "Two novel mutations in the COLQ gene cause endplate acetylcholinesterase deficiency." dans: Neuromuscular disorders : NMD, Vol. 13, Issue 3, pp. 236-44, (2003) (PubMed).

Détails sur COLQ

Antigène: COLQ (AChE Q Subunit (COLQ))

Autre désignation: COLQ (COLQ Produits)

Synonymes: anticorps EAD, anticorps A130034K24Rik, anticorps collagen like tail subunit of asymmetric acetylcholinesterase, anticorps collagen-like tail subunit (single strand of homotrimer) of asymmetric acetylcholinesterase, anticorps COLQ, anticorps Colq

Sujet: This gene encodes the subunit of a collagen-like molecule associated with acetylcholinesterase in skeletal muscle. Each molecule is composed of three identical subunits. Each subunit contains a proline-rich attachment domain (PRAD) that binds an acetylcholinesterase tetramer to anchor the catalytic subunit of the enzyme to the basal lamina. Mutations in this gene are associated with endplate acetylcholinesterase deficiency. Multiple transcript variants encoding different isoforms have been found for this gene.

Poids moléculaire: 47766

NCBI Accession: NP_005668, NP_536799, NP_536800

UniProt: Q9Y215