Rabbit anti-Human ERCC6 Polyclonal Antibody | anti-ERCC6 antibody
ERCC6 Polyclonal Conjugated Antibody
AF350 conjugated: most applications: 1:50-1:250
AF405 conjugated: most applications: 1:50-1:250
AF488 conjugated: most applications: 1:50-1:250
AF555 conjugated: most applications: 1:50-1:250
AF594 conjugated: most applications: 1:50-1:250
AF647 conjugated: most applications: 1:50-1:250
AF680 conjugated: most applications: 1:50-1:250
AF750 conjugated: most applications: 1:50-1:250
Biotin conjugated: working with enzyme-conjugated streptavidin, most applications: 1:50-1:1000
NCBI and Uniprot Product Information
NCBI Description
This gene encodes a DNA-binding protein that is important in transcription-coupled excision repair. The encoded protein has ATP-stimulated ATPase activity, interacts with several transcription and excision repair proteins, and may promote complex formation at DNA repair sites. Mutations in this gene are associated with Cockayne syndrome type B and cerebrooculofacioskeletal syndrome 1. Naturally-occurring readthrough transcription occurs between this gene and the adjacent PGBD3 gene (GeneID:267004), and results in a fusion protein that shares sequence with the product of each individual gene. The readthrough locus is represented by GeneID:101243544. [provided by RefSeq, Mar 2013]
Uniprot Description
ERCC6: 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. Defects in ERCC6 are the cause of Cockayne syndrome type B (CSB). 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); 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); 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. Defects in ERCC6 are the cause of susceptibility to age- related macular degeneration type 5 (ARMD5). A form of age-related macular degeneration, a multifactorial eye disease and the most common cause of irreversible vision loss in the developed world. In most patients, the disease is manifest as ophthalmoscopically visible yellowish accumulations of protein and lipid that lie beneath the retinal pigment epithelium and within an elastin-containing structure known as Bruch membrane. Defects in ERCC6 are a cause of UV-sensitive syndrome type 1 (UVSS1). A rare autosomal recessive disorder characterized by photosensitivity and mild freckling but without neurological abnormalities or skin tumors. Patient exhibit a number of freckles, hypopigmented spots, telangiectases, and slightly dried skin in sun-exposed areas. Belongs to the SNF2/RAD54 helicase family.
Protein type: DNA repair, damage; EC 3.6.1.-; EC 3.6.4.-; Helicase; Transcription regulation
Chromosomal Location of Human Ortholog: 10q11.23
Cellular Component: nucleolus; nucleoplasm; nucleus; transcription elongation factor complex
Molecular Function: ATP binding; chromatin binding; DNA binding; DNA helicase activity; DNA-dependent ATPase activity; protein binding; protein C-terminus binding; protein complex binding; protein N-terminus binding; protein tyrosine kinase activator activity
Biological Process: base-excision repair; positive regulation of gene expression, epigenetic; positive regulation of RNA elongation; regulation of RNA elongation; response to oxidative stress; response to UV; transcription-coupled nucleotide-excision repair
Disease: Cerebrooculofacioskeletal Syndrome 1; Cockayne Syndrome B; De Sanctis-cacchione Syndrome; Lung Cancer; Macular Degeneration, Age-related, 5; Premature Ovarian Failure 11; Uv-sensitive Syndrome 1
Research Articles on ERCC6
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Product Notes
The ERCC6 ercc6 (Catalog #AAA9441539) is an Antibody produced from Rabbit and is intended for research purposes only. The product is available for immediate purchase. The ERCC6 Polyclonal Conjugated Antibody reacts with Human and may cross-react with other species as described in the data sheet. AAA Biotech's ERCC6 can be used in a range of immunoassay formats including, but not limited to, Most Applications. Suggested Dilution: AF350 conjugated: most applications: 1:50-1:250 AF405 conjugated: most applications: 1:50-1:250 AF488 conjugated: most applications: 1:50-1:250 AF555 conjugated: most applications: 1:50-1:250 AF594 conjugated: most applications: 1:50-1:250 AF647 conjugated: most applications: 1:50-1:250 AF680 conjugated: most applications: 1:50-1:250 AF750 conjugated: most applications: 1:50-1:250 Biotin conjugated: working with enzyme-conjugated streptavidin, most applications: 1:50-1:1000. Researchers should empirically determine the suitability of the ERCC6 ercc6 for an application not listed in the data sheet. Researchers commonly develop new applications and it is an integral, important part of the investigative research process. It is sometimes possible for the material contained within the vial of "ERCC6, Polyclonal Antibody" to become dispersed throughout the inside of the vial, particularly around the seal of said vial, during shipment and storage. We always suggest centrifuging these vials to consolidate all of the liquid away from the lid and to the bottom of the vial prior to opening. Please be advised that certain products may require dry ice for shipping and that, if this is the case, an additional dry ice fee may also be required.Precautions
All products in the AAA Biotech catalog are strictly for research-use only, and are absolutely not suitable for use in any sort of medical, therapeutic, prophylactic, in-vivo, or diagnostic capacity. By purchasing a product from AAA Biotech, you are explicitly certifying that said products will be properly tested and used in line with industry standard. AAA Biotech and its authorized distribution partners reserve the right to refuse to fulfill any order if we have any indication that a purchaser may be intending to use a product outside of our accepted criteria.Disclaimer
Though we do strive to guarantee the information represented in this datasheet, AAA Biotech cannot be held responsible for any oversights or imprecisions. AAA Biotech reserves the right to adjust any aspect of this datasheet at any time and without notice. It is the responsibility of the customer to inform AAA Biotech of any product performance issues observed or experienced within 30 days of receipt of said product. To see additional details on this or any of our other policies, please see our Terms & Conditions page.Item has been added to Shopping Cart
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