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ERCC6 elisa kit :: Human excision repair cross-complementing rodent repair deficiency, complementation group 6 ELISA Kit

Scan QR to view Datasheet Catalog #    MBS9328001 ERCC6 elisa kit
Unit / Price
48-Strip-Wells  /  $435 +1 FREE 8GB USB
96-Strip-Wells  /  $600 +1 FREE 8GB USB
5x96-Strip-Wells  /  $2,650 +3 FREE 8GB USB
10x96-Strip-Wells  /  $5,205 +6 FREE 8GB USB
 
 Go to:   rightarrow  Product Names   rightarrow Product Info   rightarrow Accession #s   rightarrow Product Desc   rightarrow Diseases/Tissues/Pathways   rightarrow Applications   rightarrow References 
 Product Name   

excision repair cross-complementing rodent repair deficiency, complementation group 6 (ERCC6), ELISA Kit

 Also Known As   

Human DNA excision repair protein ERCC-6, ERCC6 ELISA Kit

 Product Synonym Names    Human DNA excision repair protein ERCC-6 (ERCC6) ELISA kit; ARMD5; CKN2; COFS; COFS1; CSB; RAD26; Cockayne syndrome B protein; Cockayne syndrome group B protein; Rad26 homolog; excision repair cross-complementing rodent repair deficiency; complementation group 6
 Product Gene Name   

ERCC6 elisa kit

[Similar Products]
 Research Use Only    For Research Use Only. Not for use in diagnostic procedures.
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 Request for Manual Insert    Request Manual
 OMIM    gene 613761
 3D Structure    ModBase 3D Structure for Q03468
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 Species Reactivity    Human
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 Preparation and Storage    Store all reagents at 2-8 degree C
 Product Note    Our ELISA assays are dynamic research tools and sometimes they may be updated and improved. If the format of this assay is important to you then please contact our technical support team with a presales inquiry before placing an order. We will confirm the current details of the assay.
 Other Notes    Small volumes of ERCC6 elisa kit vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.
 Searchable Terms for ERCC6 purchase    MBS9328001 is a ready-to-use microwell, strip plate ELISA (enzyme-linked immunosorbent assay) Kit for analyzing the presence of the excision repair cross-complementing rodent repair deficiency, complementation group 6 (ERCC6) ELISA Kit target analytes in biological samples. The concentration gradients of the kit standards or positive controls render a theoretical kit detection range in biological research samples containing ERCC6. The ELISA analytical biochemical technique of the MBS9328001 kit is based on ERCC6 antibody-ERCC6 antigen interactions (immunosorbency) and an HRP colorimetric detection system to detect ERCC6 antigen targets in samples. The ELISA Kit is designed to detect native, not recombinant, ERCC6. Appropriate sample types may include undiluted body fluids and/or tissue homogenates, secretions. Quality control assays assessing reproducibility identified the intra-assay CV (%) and inter-assay CV(%).
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NCBI/Uniprot data below describe general gene information for ERCC6. It may not necessarily be applicable to this product.
 NCBI GI #    4557565
 NCBI GeneID    2074
 NCBI Accession #    NP_000115.1 [Other Products]
 NCBI GenBank Nucleotide #    NM_000124.3 [Other Products]
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 UniProt Primary Accession #    Q03468 [Other Products]
 UniProt Secondary Accession #    Q5W0L9; D3DX94 [Other Products]
 UniProt Related Accession #    Q03468 [Other Products]
 Molecular Weight    168,416 Da
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 NCBI Official Full Name    DNA excision repair protein ERCC-6
 NCBI Official Synonym Full Names    excision repair cross-complementation group 6
 NCBI Official Symbol    ERCC6 [Similar Products]
 NCBI Official Synonym Symbols   
CSB; CKN2; COFS; ARMD5; COFS1; RAD26; UVSS1
[Similar Products]
 NCBI Protein Information    DNA excision repair protein ERCC-6; ATP-dependent helicase ERCC6; cockayne syndrome protein CSB; Cockayne syndrome group B protein; excision repair cross-complementing rodent repair deficiency, complementation group 6
 UniProt Protein Name    DNA excision repair protein ERCC-6
 UniProt Synonym Protein Names   
ATP-dependent helicase ERCC6; Cockayne syndrome protein CSB
 Protein Family    DNA excision repair protein
 UniProt Gene Name    ERCC6 [Similar Products]
 UniProt Synonym Gene Names    CSB [Similar Products]
 UniProt Entry Name    ERCC6_HUMAN
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 NCBI Summary for ERCC6    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]
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 UniProt Comments for ERCC6    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.-; Helicase; EC 3.6.4.-; Transcription regulation

Chromosomal Location of Human Ortholog: 10q11.23

Cellular Component: nucleoplasm; transcription elongation factor complex; nucleolus; nucleus

Molecular Function: protein C-terminus binding; DNA-dependent ATPase activity; DNA helicase activity; protein binding; DNA binding; protein complex binding; protein tyrosine kinase activator activity; protein N-terminus binding; chromatin binding; ATP binding

Biological Process: transcription from RNA polymerase II promoter; response to superoxide; positive regulation of RNA elongation; multicellular organism growth; response to toxin; photoreceptor cell maintenance; RNA elongation from RNA polymerase I promoter; DNA repair; activation of JNK activity; response to UV-B; DNA damage response, signal transduction resulting in induction of apoptosis; base-excision repair; nucleotide-excision repair; transcription-coupled nucleotide-excision repair; response to gamma radiation; pyrimidine dimer repair; response to oxidative stress; regulation of RNA elongation; response to X-ray; activation of JNKK activity; response to UV

Disease: Lung Cancer; Cockayne Syndrome B; Macular Degeneration, Age-related, 5; Uv-sensitive Syndrome 1; Cerebrooculofacioskeletal Syndrome 1; De Sanctis-cacchione Syndrome
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 Research Articles on ERCC6    1. ERCC6 rs1917799 polymorphism is associated with gastric cancer risk.
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 Precautions    All of MyBioSource's Products are for scientific laboratory research purposes and are not for diagnostic, therapeutics, prophylactic or in vivo use. Through your purchase, you expressly represent and warrant to MyBioSource that you will properly test and use any Products purchased from MyBioSource in accordance with industry standards. MyBioSource and its authorized distributors reserve the right to refuse to process any order where we reasonably believe that the intended use will fall outside of our acceptable guidelines.
 Disclaimer    While every efforts were made to ensure the accuracy of the information provided in this datasheet, MyBioSource will not be liable for any omissions or errors contained herein. MyBioSource reserves the right to make changes to this datasheet at any time without prior notice.

It is the responsibility of the customer to report product performance issues to MyBioSource within 30 days of receipt of the product. Please visit our Terms & Conditions page for more information.
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Products associated with ERCC6 elisa kitPathways associated with ERCC6 elisa kit
 Reference Product  PubMed Publications
 ERCC8 elisa kit  >40 publications with ERCC6 and ERCC8
 ERCC2 elisa kit  >32 publications with ERCC6 and ERCC2
 ERCC5 elisa kit  >20 publications with ERCC6 and ERCC5
 ERCC4 elisa kit  >15 publications with ERCC6 and ERCC4
 ERCC3 elisa kit  >11 publications with ERCC6 and ERCC3
 TP53 elisa kit  >8 publications with ERCC6 and TP53
 XAB2 elisa kit  >2 publications with ERCC6 and XAB2
 Products by Pathway  Pathway Diagram
 DNA Repair Pathway antibodies  DNA Repair Pathway Diagram
 Dual Incision Reaction In TC-NER Pathway antibodies  Dual Incision Reaction In TC-NER Pathway Diagram
 Formation Of Transcription-coupled NER (TC-NER) Repair Complex Pathway antibodies  Formation Of Transcription-coupled NER (TC-NER) Repair Complex Pathway Diagram
 Gene Expression Pathway antibodies  Gene Expression Pathway Diagram
 Nucleotide Excision Repair Pathway antibodies  Nucleotide Excision Repair Pathway Diagram
 Nucleotide Excision Repair Pathway antibodies  Nucleotide Excision Repair Pathway Diagram
 Nucleotide Excision Repair Pathway antibodies  Nucleotide Excision Repair Pathway Diagram
 RNA Polymerase I Promoter Clearance Pathway antibodies  RNA Polymerase I Promoter Clearance Pathway Diagram
 RNA Polymerase I Transcription Pathway antibodies  RNA Polymerase I Transcription Pathway Diagram
 RNA Polymerase I Transcription Initiation Pathway antibodies  RNA Polymerase I Transcription Initiation Pathway Diagram
Diseases associated with ERCC6 elisa kitOrgans/Tissues associated with ERCC6 elisa kit
 Disease Name  Pubmed Publications
 Cockayne Syndrome Antibodies  >169 publications with ERCC6 and Cockayne Syndrome
 Nervous System Diseases Antibodies  >122 publications with ERCC6 and Nervous System Diseases
 Death Antibodies  >15 publications with ERCC6 and Death
 Carcinoma Antibodies  >13 publications with ERCC6 and Carcinoma
 Disease Models, Animal Antibodies  >10 publications with ERCC6 and Disease Models, Animal
 Microcephaly Antibodies  >9 publications with ERCC6 and Microcephaly
 Lung Diseases Antibodies  >8 publications with ERCC6 and Lung Diseases
 Growth Disorders Antibodies  >7 publications with ERCC6 and Growth Disorders
 Lung Neoplasms Antibodies  >7 publications with ERCC6 and Lung Neoplasms
 Cataract Antibodies  >6 publications with ERCC6 and Cataract
 Organ/Tissue Name  Pubmed Publications
 Skin Antibodies  >25 publications with ERCC6 and Skin
 Brain Antibodies  >24 publications with ERCC6 and Brain
 Blood Antibodies  >16 publications with ERCC6 and Blood
 Liver Antibodies  >14 publications with ERCC6 and Liver
 Lung Antibodies  >11 publications with ERCC6 and Lung
 Eye Antibodies  >9 publications with ERCC6 and Eye
 Kidney Antibodies  >7 publications with ERCC6 and Kidney
 Prostate Antibodies  >5 publications with ERCC6 and Prostate
 Vascular Antibodies  >4 publications with ERCC6 and Vascular
 Bladder Antibodies  >4 publications with ERCC6 and Bladder
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