Anti-CSB antibody (ab87835)
Key features and details
- Rabbit polyclonal to CSB
- Suitable for: WB
- Reacts with: Human
- Isotype: IgG
Overview
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Product name
Anti-CSB antibody
See all CSB primary antibodies -
Description
Rabbit polyclonal to CSB -
Host species
Rabbit -
Tested Applications & Species
See all applications and species dataApplication Species WB Human -
Immunogen
Synthetic peptide corresponding to Human CSB aa 200-300 conjugated to keyhole limpet haemocyanin.
(Peptide available asab114005) -
Positive control
- This antibody gave a positive signal in the following lysates: HeLa Whole Cell Lysate - Irradiated (5Gy).
Properties
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Form
Liquid -
Storage instructions
Shipped at 4°C. Store at +4°C short term (1-2 weeks). Upon delivery aliquot. Store at -20°C or -80°C. Avoid freeze / thaw cycle. -
Storage buffer
pH: 7.40
Preservative: 0.02% Sodium azide
Constituent: PBS
Batches of this product that have a concentrationConcentration information loading...Purity
Immunogen affinity purifiedClonality
PolyclonalIsotype
IgGResearch areas
Associated products
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Compatible Secondaries
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Isotype control
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Recombinant Protein
Applications
The Abpromise guarantee
Our Abpromise guarantee covers the use of ab87835 in the following tested applications.
The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
GuaranteedTested applications are guaranteed to work and covered by our Abpromise guarantee.
PredictedPredicted to work for this combination of applications and species but not guaranteed.
IncompatibleDoes not work for this combination of applications and species.
Application Species WB HumanAll applications ChickenCowDogApplication Abreviews Notes WB Use a concentration of 1 µg/ml. Detects a band of approximately 175 kDa (predicted molecular weight: 168 kDa).Notes WB
Use a concentration of 1 µg/ml. Detects a band of approximately 175 kDa (predicted molecular weight: 168 kDa).Target
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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
modificationsPhosphorylated 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. - Information by UniProt
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Database links
- Entrez Gene: 423785 Chicken
- Entrez Gene: 522908 Cow
- Entrez Gene: 477747 Dog
- Entrez Gene: 2074 Human
- Omim: 609413 Human
- SwissProt: Q03468 Human
- Unigene: 654449 Human
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Alternative names
- 4732403I04 antibody
- ARMD 5 antibody
- ARMD5 antibody
see all
Images
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Anti-CSB antibody (ab87835) at 1 µg/ml + HeLa Whole Cell Lysate - Irradiated (5Gy) at 10 µg
Secondary
Goat Anti-Rabbit IgG H&L (HRP) preadsorbed (ab97080) at 1/5000 dilution
Developed using the ECL technique.
Performed under reducing conditions.
Predicted band size: 168 kDa
Observed band size: 175 kDa why is the actual band size different from the predicted?
Additional bands at: 300 kDa, 50 kDa. We are unsure as to the identity of these extra bands.
Exposure time: 150 seconds
Abcam recommends loading unheated lysates and using 20% Ethanol and 0.04% SDS in the transfer buffer to obtain a positive signal for this antibody. Abcam welcomes customer feedback and would appreciate any comments regarding this product and the data presented above
Datasheets and documents
References (0)
ab87835 has not yet been referenced specifically in any publications.
Images
-
Anti-CSB antibody (ab87835) at 1 µg/ml + HeLa Whole Cell Lysate - Irradiated (5Gy) at 10 µg
Secondary
Goat Anti-Rabbit IgG H&L (HRP) preadsorbed (ab97080) at 1/5000 dilution
Developed using the ECL technique.
Performed under reducing conditions.
Predicted band size: 168 kDa
Observed band size: 175 kDa why is the actual band size different from the predicted?
Additional bands at: 300 kDa, 50 kDa. We are unsure as to the identity of these extra bands.
Exposure time: 150 seconds
Abcam recommends loading unheated lysates and using 20% Ethanol and 0.04% SDS in the transfer buffer to obtain a positive signal for this antibody. Abcam welcomes customer feedback and would appreciate any comments regarding this product and the data presented above