製品の概要

  • 製品名Anti-CSB antibody
    CSB 一次抗体 製品一覧
  • 製品の詳細
    Goat polyclonal to CSB
  • アプリケーション適用あり: IHC-Pmore details
  • 種交差性
    交差種: Human
  • 免疫原

    Synthetic peptide:

    SGGEGIWKLKPEYC

    , corresponding to amino acids 1480-1493 of Human CSB.

  • ポジティブ・コントロール
    • Human Colon and Small Intestine tissues

製品の特性

  • 製品の状態Liquid
  • 保存方法Shipped at 4°C. Upon delivery aliquot and store at -20°C. Avoid freeze / thaw cycles.
  • バッファーpH: 7.30
    Preservative: 0.02% Sodium azide
    Constituents: 99% Tris buffered saline, 0.5% BSA
  • Concentration information loading...
  • 精製度Immunogen affinity purified
  • ポリ/モノポリクローナル
  • アイソタイプIgG
  • 研究分野

アプリケーション

Our Abpromise guarantee covers the use of ab118562 in the following tested applications.

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

アプリケーション Abreviews 特記事項
IHC-P Use a concentration of 3.75 - 5 µg/ml.

ターゲット情報

  • 機能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.
  • 関連疾患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.
  • 配列類似性Belongs to the SNF2/RAD54 helicase family.
    Contains 1 helicase ATP-binding domain.
    Contains 1 helicase C-terminal domain.
  • ドメインA C-terminal ubiquitin-binding domain (UBD) is essential for transcription-coupled nucleotide excision repair to proceed.
  • 翻訳後修飾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.
  • 細胞内局在Nucleus.
  • Information by UniProt
  • 参照データベース
  • 別名
    • 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 画像

  • ab118562, at 5 µg/ml, staining CSB in formalin-fixed, paraffin-embedded Human Colon tissue by Immunohistochemistry.
  • ab118562, at 5 µg/ml, staining CSB in formalin-fixed, paraffin-embedded Human Small Intestine tissue by Immunohistochemistry.

Anti-CSB antibody (ab118562) 使用論文

ab118562 has not yet been referenced specifically in any publications.

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