Anti-CSB抗体(ab96089)

概述

  • 产品名称Anti-CSB抗体
    参阅全部 CSB 一抗
  • 描述
    兔多克隆抗体to CSB
  • 经测试应用适用于: WB, ICC/IFmore details
  • 种属反应性
    与反应: Human
    预测可用于: Mouse
  • 免疫原

    Recombinant protein fragment contain a sequence corresponding to a region within amino acids 346 and 736 of Human CSB

  • 阳性对照
    • A431 whole cell lysate and in culture. H1299 whole cell lysate. 293T, H1299, and HepG2.

性能

  • 形式Liquid
  • 存放说明Shipped at 4°C. Upon delivery aliquot and store at -20°C or -80°C. Avoid repeated freeze / thaw cycles.
  • 存储溶液Preservative: 0.01% Thimerosal (merthiolate)
    Constituents: 10% Glycerol, 0.1M Tris, 0.1M Glycine, pH 7
  • Concentration information loading...
  • 纯度Immunogen affinity purified
  • 克隆多克隆
  • 同种型IgG
  • 研究领域

相关产品

应用

Our Abpromise guarantee covers the use of ab96089 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 1/500 - 1/3000. Predicted molecular weight: 168 kDa.
ICC/IF 1/100 - 1/200.

靶标

  • 功能Essential factor involved in transcription-coupled nucleotide excision repair which allows RNA polymerase II-blocking lesions to be rapidly removed from the transcribed strand of active genes. Upon DNA-binding, it locally modifies DNA conformation by wrapping the DNA around itself, thereby modifying the interface between stalled RNA polymerase II and DNA. It is required for transcription-coupled repair complex formation. It recruits the CSA complex (DCX(ERCC8) complex), nucleotide excision repair proteins and EP300 to the at sites of RNA polymerase II-blocking lesions.
  • 疾病相关Defects in ERCC6 are the cause of Cockayne syndrome type B (CSB) [MIM:133540]. Cockayne syndrome is a rare disorder characterized by cutaneous sensitivity to sunlight, abnormal and slow growth, cachectic dwarfism, progeroid appearance, progressive pigmentary retinopathy and sensorineural deafness. There is delayed neural development and severe progressive neurologic degeneration resulting in mental retardation. Two clinical forms are recognized: in the classical form or Cockayne syndrome type 1, the symptoms are progressive and typically become apparent within the first few years or life; the less common Cockayne syndrome type 2 is characterized by more severe symptoms that manifest prenatally. Cockayne syndrome shows some overlap with certain forms of xeroderma pigmentosum. Unlike xeroderma pigmentosum, patients with Cockayne syndrome do not manifest increased freckling and other pigmentation abnormalities in the skin and have no significant increase in skin cancer.
    Defects in ERCC6 are the cause of cerebro-oculo-facio-skeletal syndrome type 1 (COFS1) [MIM:214150]; also known as COFS syndrome or Pena-Shokeir syndrome type 2. COFS is a degenerative autosomal recessive disorder of prenatal onset affecting the brain, eye and spinal cord. After birth, it leads to brain atrophy, hypoplasia of the corpus callosum, hypotonia, cataracts, microcornea, optic atrophy, progressive joint contractures and growth failure. Facial dysmorphism is a constant feature. Abnormalities of the skull, eyes, limbs, heart and kidney also occur.
    Defects in ERCC6 are a cause of De Sanctis-Cacchione syndrome (DSC) [MIM:278800]; also known as xerodermic idiocy. DSC is an autosomal recessive syndrome consisting of xeroderma pigmentosum associated with mental retardation, retarded growth, gonadal hypoplasia and sometimes neurologic complications.
    Note=A genetic variation in the 5-prime flanking region of ERCC6 has been shown to be associated with susceptibility to age-related macular degeneration.
    Defects in ERCC6 are a cause of UV-sensitive syndrome (UVS) [MIM:600630]. UVS is a rare autosomal recessive disorder characterized by photosensitivity and mild freckling but without neurological abnormalities or skin tumors.
  • 序列相似性Belongs to the SNF2/RAD54 helicase family.
    Contains 1 helicase ATP-binding domain.
    Contains 1 helicase C-terminal domain.
  • 结构域A C-terminal ubiquitin-binding domain (UBD) is essential for transcription-coupled nucleotide excision repair to proceed.
  • 翻译后修饰Phosphorylated upon DNA damage, probably by ATM or ATR.
    Ubiquitinated at the C-terminus. Ubiquitination by the CSA complex leads to ERCC6 proteasomal degradation in a UV-dependent manner.
  • 细胞定位Nucleus.
  • Information by UniProt
  • 数据库链接
  • 别名
    • 4732403I04 antibody
    • ARMD 5 antibody
    • ARMD5 antibody
    • ATP dependent helicase ERCC6 antibody
    • ATP-dependent helicase ERCC6 antibody
    • C130058G22Rik antibody
    • CKN 2 antibody
    • CKN2 antibody
    • Cockayne syndrome B protein antibody
    • Cockayne syndrome group B protein antibody
    • Cockayne syndrome protein CSB antibody
    • COFS antibody
    • COFS1 antibody
    • CS group B correcting antibody
    • CSB antibody
    • DNA excision repair protein ERCC 6 antibody
    • DNA excision repair protein ERCC-6 antibody
    • ERCC 6 antibody
    • ERCC6 antibody
    • ERCC6_HUMAN antibody
    • Excision repair cross complementing rodent repair deficiency, complementation group 6 antibody
    • OTTHUMP00000019581 antibody
    • RAD26 antibody
    • Rad26 homolog antibody
    • UVSS1 antibody
    see all

Anti-CSB antibody 图像

  • All lanes : Anti-CSB antibody (ab96089) at 1/500 dilution

    Lane 1 : A431 whole cell lysate
    Lane 2 : H1299 whole cell lysate

    Lysates/proteins at 30 µg per lane.


    Predicted band size : 168 kDa
  • Immunofluorescence analysis of paraformaldehyde-fixed A431, using ab96089 antibody at 1/200 dilution. Lower image merged with DNA probe.

Anti-CSB antibody (ab96089)参考文献

ab96089 has not yet been referenced specifically in any publications.

Product Wall

Application Western blot
Loading amount 30 µg
Gel Running Conditions Reduced Denaturing (4-12%)
Sample Human Cell lysate - whole cell (lymphocytes)
Specification lymphocytes
Blocking step BSA as blocking agent for 1 hour(s) and 0 minute(s) · Concentration: 2% · Temperature: 25°C
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提交于 Aug 06 2014

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