COLQ Antikörper (C-Term)
Kurzübersicht für COLQ Antikörper (C-Term) (ABIN1881222)
Target
Alle COLQ Antikörper anzeigenReaktivität
Wirt
Klonalität
Konjugat
Applikation
Klon
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Bindungsspezifität
- AA 324-353, C-Term
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Homologie
- Rat
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Aufreinigung
- This antibody is purified through a protein A column, followed by peptide affinity purification.
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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.
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Isotyp
- Ig Fraction
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Applikationshinweise
- WB: 1:1000
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Beschränkungen
- Nur für Forschungszwecke einsetzbar
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Format
- Liquid
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Buffer
- Purified polyclonal antibody supplied in PBS with 0.09 % (W/V) sodium azide.
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Konservierungsmittel
- Sodium azide
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Vorsichtsmaßnahmen
- This product contains Sodium azide: a POISONOUS AND HAZARDOUS SUBSTANCE which should be handled by trained staff only.
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Lagerung
- 4 °C,-20 °C
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Haltbarkeit
- 6 months
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: "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).
: "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).
: "Two novel mutations in the COLQ gene cause endplate acetylcholinesterase deficiency." in: Neuromuscular disorders : NMD, Vol. 13, Issue 3, pp. 236-44, (2003) (PubMed).
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- COLQ (AChE Q Subunit (COLQ))
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Andere Bezeichnung
- COLQ
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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.
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Molekulargewicht
- 47766
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NCBI Accession
- NP_005668, NP_536799, NP_536800
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UniProt
- Q9Y215
Target
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