COLQ Antikörper (C-Term)

Produktnummer ABIN1881222

Produktdetails anti-COLQ Antikörper

Target: COLQ (AChE Q Subunit (COLQ))

Bindungsspezifität: AA 324-353, C-Term

Reaktivität: Human, Maus

Wirt: Kaninchen

Klonalität: Polyklonal

Konjugat: Dieser COLQ Antikörper ist unkonjugiert

Applikation: Western Blotting (WB)

Homologie: Rat

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

Immunogen: 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.

Klon: RB30589

Isotyp: Ig Fraction

Anwendungsinformationen

Applikationshinweise: WB: 1:1000

Beschränkungen: Nur für Forschungszwecke einsetzbar

Handhabung

Format: Liquid

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

Konservierungsmittel: Sodium azide

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

Lagerung: 4 °C,-20 °C

Haltbarkeit: 6 months

Referenzen für anti-COLQ Antikörper (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." in: 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." in: 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." in: Neuromuscular disorders : NMD, Vol. 13, Issue 3, pp. 236-44, (2003) (PubMed).

Details zu COLQ

Target: COLQ (AChE Q Subunit (COLQ))

Andere Bezeichnung: COLQ (COLQ Produkte)

Hintergrund: 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.

Molekulargewicht: 47766

NCBI Accession: NP_005668, NP_536799, NP_536800

UniProt: Q9Y215