Key features and details
- Rabbit polyclonal to CSB
- Suitable for: WB, ICC/IF
- Reacts with: Human
- Isotype: IgG
参阅全部 CSB 一抗
经测试应用适用于: WB, ICC/IFmore details
Recombinant fragment within Human CSB aa 300-750. The exact immunogen sequence used to generate this antibody is proprietary information. If additional detail on the immunogen is needed to determine the suitability of the antibody for your needs, please contact our Scientific Support team to discuss your requirements.
- A431 whole cell lysate and in culture. H1299 whole cell lysate. 293T, H1299, and HepG2.
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存放说明Shipped at 4°C. Upon delivery aliquot and store at -20°C or -80°C. Avoid repeated freeze / thaw cycles.
Preservative: 0.025% Proclin 300
Constituents: 50% Glycerol (glycerin, glycerine), 2.4% Tris, 1.5% Glycine, 46% PBS, 0.04% EGTA
Concentration information loading...
纯度Immunogen affinity purified
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.
- Information by UniProt
- 4732403I04 antibody
- ARMD 5 antibody
- ARMD5 antibody
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
7.5% SDS Page
Immunofluorescence analysis of paraformaldehyde-fixed A431, using ab96089 antibody at 1/200 dilution. Lower image merged with DNA probe.
ab96089 被引用在 6 文献中.
- Mulderrig L & Garaycoechea JI XPF-ERCC1 protects liver, kidney and blood homeostasis outside the canonical excision repair pathways. PLoS Genet 16:e1008555 (2020). PubMed: 32271760
- Tan J et al. An R-loop-initiated CSB-RAD52-POLD3 pathway suppresses ROS-induced telomeric DNA breaks. Nucleic Acids Res 48:1285-1300 (2020). PubMed: 31777915
- Crochemore C et al. CSB promoter downregulation via histone H3 hypoacetylation is an early determinant of replicative senescence. Nat Commun 10:5576 (2019). PubMed: 31811121
- Lee JH et al. Cockayne syndrome group B deficiency reduces H3K9me3 chromatin remodeler SETDB1 and exacerbates cellular aging. Nucleic Acids Res 47:8548-8562 (2019). PubMed: 31276581
- Yang Z et al. CSB affected on the sensitivity of lung cancer cells to platinum-based drugs through the global decrease of let-7 and miR-29. BMC Cancer 19:948 (2019). PubMed: 31615563
- Iyama T et al. CSB interacts with SNM1A and promotes DNA interstrand crosslink processing. Nucleic Acids Res 43:247-58 (2015). WB . PubMed: 25505141