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    csb-antibody-553c5a-ab66598.pdf

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Epigenetics and Nuclear Signaling DNA / RNA DNA Damage & Repair Nucl. Excision Repair
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Anti-CSB antibody [553C5a] (ab66598)

  • Datasheet
Submit a review Submit a question References (3)

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Abpromise

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Flow Cytometry - Anti-CSB antibody [553C5a] (ab66598)
  • Western blot - Anti-CSB antibody [553C5a] (ab66598)

Key features and details

  • Mouse monoclonal [553C5a] to CSB
  • Suitable for: Flow Cyt, WB
  • Reacts with: Human
  • Isotype: IgG2b

You may also be interested in

Protein
Product image
Recombinant Human CSB protein (ab114506)
Secondary
Product image
Goat Anti-Mouse IgG H&L (HRP) (ab205719)

View more associated products

Overview

  • Product name

    Anti-CSB antibody [553C5a]
    See all CSB primary antibodies
  • Description

    Mouse monoclonal [553C5a] to CSB
  • Host species

    Mouse
  • Tested applications

    Suitable for: Flow Cyt, WBmore details
  • Species reactivity

    Reacts with: Human
  • Immunogen

    Recombinant fragment corresponding to an internal region carrying 50-200 aa of Human CSB

  • General notes

    Reproducibility is key to advancing scientific discovery and accelerating scientists’ next breakthrough.

    Abcam is leading the way with our range of recombinant antibodies, knockout-validated antibodies and knockout cell lines, all of which support improved reproducibility.

    We are also planning to innovate the way in which we present recommended applications and species on our product datasheets, so that only applications & species that have been tested in our own labs, our suppliers or by selected trusted collaborators are covered by our Abpromise™ guarantee.

    In preparation for this, we have started to update the applications & species that this product is Abpromise guaranteed for.

    We are also updating the applications & species that this product has been “predicted to work with,” however this information is not covered by our Abpromise guarantee.

    Applications & species from publications and Abreviews that have not been tested in our own labs or in those of our suppliers are not covered by the Abpromise guarantee.

    Please check that this product meets your needs before purchasing. If you have any questions, special requirements or concerns, please send us an inquiry and/or contact our Support team ahead of purchase. Recommended alternatives for this product can be found below, as well as customer reviews and Q&As.

Properties

  • Form

    Liquid
  • Storage instructions

    Shipped at 4°C. Upon delivery aliquot and store at -20°C or -80°C. Avoid repeated freeze / thaw cycles.
  • Storage buffer

    pH: 7.40
    Preservative: 0.05% Sodium azide
    Constituents: PBS, 0.0225% Potassium chloride, 0.03% Potassium phosphate, 0.1312% Sodium phosphate, 0.812% Sodium chloride, 1% BSA
  • Concentration information loading...
  • Purity

    Protein G purified
  • Purification notes

    Purified from culture supernatant of hybridoma cultured in a medium containing bovine IgG-depleted (approximately 95%) fetal bovine serum and filtered through a 0.22 µm membrane.
  • Clonality

    Monoclonal
  • Clone number

    553C5a
  • Isotype

    IgG2b
  • Research areas

    • Epigenetics and Nuclear Signaling
    • DNA / RNA
    • DNA Damage & Repair
    • Nucl. Excision Repair

Associated products

  • Compatible Secondaries

    • Goat Anti-Mouse IgG H&L (Alexa Fluor® 488) (ab150113)
    • Goat Anti-Mouse IgG H&L (HRP) (ab205719)
  • Isotype control

    • Mouse IgG2b, kappa monoclonal [7E10G10] - Isotype Control (ab170192)
  • Recombinant Protein

    • Recombinant Human CSB protein (ab114506)

Applications

Our Abpromise guarantee covers the use of ab66598 in the following tested applications.

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

Application Abreviews Notes
Flow Cyt Use 1µg for 106 cells.

ab170192 - Mouse monoclonal IgG2b, is suitable for use as an isotype control with this antibody.

 

WB 1/100. Predicted molecular weight: 168 kDa.

Target

  • Function

    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.
  • Involvement in disease

    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.
  • Sequence similarities

    Belongs to the SNF2/RAD54 helicase family.
    Contains 1 helicase ATP-binding domain.
    Contains 1 helicase C-terminal domain.
  • Domain

    A C-terminal ubiquitin-binding domain (UBD) is essential for transcription-coupled nucleotide excision repair to proceed.
  • Post-translational
    modifications

    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.
  • Cellular localization

    Nucleus.
  • Target information above from: UniProt accession Q03468 The UniProt Consortium
    The Universal Protein Resource (UniProt) in 2010
    Nucleic Acids Res. 38:D142-D148 (2010) .

    Information by UniProt
  • Database links

    • Entrez Gene: 2074 Human
    • Omim: 609413 Human
    • SwissProt: Q03468 Human
    • Unigene: 654449 Human
    • Alternative names

      • 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
      • ERCC excision repair 6 chromatin remodeling factor 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

    Images

    • Flow Cytometry - Anti-CSB antibody [553C5a] (ab66598)
      Flow Cytometry - Anti-CSB antibody [553C5a] (ab66598)
      Overlay histogram showing HeLa cells stained with ab66598 (red line). The cells were fixed with 80% methanol (5 min) and then permeabilized with 0.1% PBS-Tween for 20 min. The cells were then incubated in 1x PBS / 10% normal goat serum / 0.3M glycine to block non-specific protein-protein interactions followed by the antibody (ab66598, 1μg/1x106 cells) for 30 min at 22°C. The secondary antibody used was Alexa Fluor® 488 goat anti-mouse IgG (H&L) (ab150113) at 1/2000 dilution for 30 min at 22°C. Isotype control antibody (black line) was mouse IgG2b [PLPV219] (ab91366, 1μg/1x106 cells) used under the same conditions. Unlabelled sample (blue line) was also used as a control. Acquisition of >5,000 events were collected using a 20mW Argon ion laser (488nm) and 525/30 bandpass filter.
    • Western blot - Anti-CSB antibody [553C5a] (ab66598)
      Western blot - Anti-CSB antibody [553C5a] (ab66598)
      Anti-CSB antibody [553C5a] (ab66598) + immunising recombinant protein

      Predicted band size: 168 kDa
      Observed band size: 45 kDa
      why is the actual band size different from the predicted?



      Primary antibody dilution 1:100, Recombinant protein amount 10ng/lane, Secondary antibody dilution 1:3000, Secondary antibody Sheep anti mouse IgG (0.63mg/ml)

    Protocols

    • Flow cytometry protocols
    • Immunocytochemistry & immunofluorescence protocols
    • Western blot protocols

    Click here to view the general protocols

    Datasheets and documents

    • Datasheet
  • References (3)

    Publishing research using ab66598? Please let us know so that we can cite the reference in this datasheet.

    ab66598 has been referenced in 3 publications.

    • Iyama T & Wilson DM Elements That Regulate the DNA Damage Response of Proteins Defective in Cockayne Syndrome. J Mol Biol 428:62-78 (2016). PubMed: 26616585
    • Iyama T  et al. CSB interacts with SNM1A and promotes DNA interstrand crosslink processing. Nucleic Acids Res 43:247-58 (2015). IP . PubMed: 25505141
    • Batenburg NL  et al. Cockayne Syndrome group B protein interacts with TRF2 and regulates telomere length and stability. Nucleic Acids Res : (2012). ICC/IF, IP ; Human . PubMed: 22904069

    Customer reviews and Q&As

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