概述

  • 产品名称Anti-CLCN2抗体
    参阅全部 CLCN2 一抗
  • 描述
    山羊多克隆抗体to CLCN2
  • 经测试应用适用于: WBmore details
  • 种属反应性
    与反应: Human
    预测可用于: Mouse, Rat, Rabbit, Horse, Cow, Dog, Pig, Monkey, Marmoset (common)
  • 免疫原

    Synthetic peptide:

    QQLDEPVNFSDCK

    , corresponding to internal sequence amino acids 777-789 of Human CLCN2 (NP_004357.3).

  • 阳性对照
    • HeLa cell lysate.

性能

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应用

Our Abpromise guarantee covers the use of ab99501 in the following tested applications.

The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.

应用 Ab评论 说明
WB Use a concentration of 0.3 - 1 µg/ml. Detects a band of approximately 100 kDa (predicted molecular weight: 98 kDa).

靶标

  • 功能Voltage-gated chloride channel. Chloride channels have several functions including the regulation of cell volume; membrane potential stabilization, signal transduction and transepithelial transport.
  • 组织特异性Ubiquitously expressed. Moderately expressed in aortic and coronary vascular smooth muscle cells and expressed at a low level in aortic endothelial cells.
  • 疾病相关Defects in CLCN2 are associated with susceptibility to idiopathic generalized epilepsy type 11 (IGE11) [MIM:607628]. A disorder characterized by recurring generalized seizures in the absence of detectable brain lesions and/or metabolic abnormalities. Generalized seizures arise diffusely and simultaneously from both hemispheres of the brain.
    Defects in CLCN2 are the cause of childhood absence epilepsy type 3 (ECA3) [MIM:607682]. ECA3 is a subtype of idiopathic generalized epilepsy (IGE) characterized by onset at age 6-7 years, frequent absence seizures (several per day) and bilateral, synchronous, symmetric 3 Hz spike waves on EEG. During adolescence, tonic-clonic and myoclonic seizures develop.
    Defects in CLCN2 are associated with juvenile absence epilepsy type 2 (JAE2) [MIM:607628]. JAE is a subtype of idiopathic generalized epilepsy (IGE) characterized by onset occurring around puberty, absence seizures, generalized tonic-clonic seizures (GTCS), GTCS on awakening and myoclonic seizures.
    Defects in CLCN2 are associated with juvenile myoclonic epilepsy type 8 (EJM8) [MIM:607628]. A subtype of idiopathic generalized epilepsy. Patients have afebrile seizures only, with onset in adolescence (rather than in childhood) and myoclonic jerks which usually occur after awakening and are triggered by sleep deprivation and fatigue.
  • 序列相似性Belongs to the chloride channel (TC 2.A.49) family. ClC-2/CLCN2 subfamily.
    Contains 2 CBS domains.
  • 细胞定位Membrane.
  • Information by UniProt
  • 数据库链接
  • 别名
    • Chloride Channel 2 antibody
    • Chloride channel protein 2 antibody
    • Chloride channel, voltage sensitive 2 antibody
    • CIC 2 antibody
    • CIC2 antibody
    • ClC-2 antibody
    • CLC2 antibody
    • Clcn2 antibody
    • CLCN2_HUMAN antibody
    • ECA2 antibody
    • ECA3 antibody
    • EG13 antibody
    • EGI11 antibody
    • EGMA antibody
    • EJM6 antibody
    • EJM8 antibody
    • PKA-activated chloride channel antibody
    see all

Anti-CLCN2 antibody 图像

  • Anti-CLCN2 antibody (ab99501) at 0.3 µg/ml + HeLa cell lysate at 35 µg
    Developed using the ECL technique

    Predicted band size : 98 kDa

Anti-CLCN2 antibody (ab99501)参考文献

ab99501 has not yet been referenced specifically in any publications.

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