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COLQ 抗体 (C-Term)

COLQ 适用: 人, 小鼠 WB 宿主: 兔 Polyclonal RB30589 unconjugated
产品编号 ABIN1881222
发货至: 中国
  • 抗原 See all COLQ 抗体
    COLQ (AChE Q Subunit (COLQ))
    抗原表位
    • 13
    • 6
    • 2
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    AA 324-353, C-Term
    适用
    • 29
    • 17
    • 10
    • 2
    • 1
    • 1
    • 1
    人, 小鼠
    宿主
    • 28
    • 1
    克隆类型
    • 29
    多克隆
    标记
    • 12
    • 3
    • 2
    • 2
    • 2
    • 2
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    This COLQ antibody is un-conjugated
    应用范围
    • 22
    • 15
    • 2
    • 1
    • 1
    • 1
    Western Blotting (WB)
    预测反应
    Rat
    纯化方法
    This antibody is purified through a protein A column, followed by peptide affinity purification.
    免疫原
    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.
    克隆位点
    RB30589
    亚型
    Ig Fraction
  • 应用备注
    WB: 1:1000
    限制
    仅限研究用
  • 状态
    Liquid
    缓冲液
    Purified polyclonal antibody supplied in PBS with 0.09 % (W/V) sodium azide.
    储存液
    Sodium azide
    注意事项
    This product contains Sodium azide: a POISONOUS AND HAZARDOUS SUBSTANCE which should be handled by trained staff only.
    储存条件
    4 °C,-20 °C
    有效期
    6 months
  • 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).

  • 抗原
    COLQ (AChE Q Subunit (COLQ))
    别名
    COLQ (COLQ 产品)
    别名
    EAD antibody, A130034K24Rik antibody, collagen like tail subunit of asymmetric acetylcholinesterase antibody, collagen-like tail subunit (single strand of homotrimer) of asymmetric acetylcholinesterase antibody, COLQ antibody, Colq antibody
    背景
    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.
    分子量
    47766
    NCBI登录号
    NP_005668, NP_536799, NP_536800
    UniProt
    Q9Y215
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