{"product_id":"yp-ab-01629-100ul","title":"CSB Polyclonal Antibody","description":"\u003cul\u003e\n\u003cli\u003e\n\u003cb\u003eGene Name:\u003c\/b\u003e ERCC6\u003c\/li\u003e\n\u003cli\u003e\n\u003cb\u003eImmunogen:\u003c\/b\u003e The antiserum was produced against synthesized peptide derived from human ERCC6. AA range:141-190\u003c\/li\u003e\n\u003cli\u003e\n\u003cb\u003eSpecificity:\u003c\/b\u003e CSB Polyclonal Antibody detects endogenous levels of CSB protein.\u003c\/li\u003e\n\u003cli\u003e\n\u003cb\u003eComposition:\u003c\/b\u003e Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide.\u003c\/li\u003e\n\u003cli\u003e\n\u003cb\u003eSource:\u003c\/b\u003e Polyclonal, Rabbit,IgG\u003c\/li\u003e\n\u003cli\u003e\n\u003cb\u003eDilution ratio:\u003c\/b\u003e IHC: 1\/100 - 1\/300. ELISA: 1\/40000.. IF 1:50-200\u003c\/li\u003e\n\u003cli\u003e\n\u003cb\u003ePurification process:\u003c\/b\u003e The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.\u003c\/li\u003e\n\u003cli\u003e\n\u003cb\u003eConcentration:\u003c\/b\u003e 1 mg\/ml\u003c\/li\u003e\n\u003cli\u003e\n\u003cb\u003eStorage:\u003c\/b\u003e -20°C\/1 year\u003c\/li\u003e\n\u003cli\u003e\n\u003cb\u003eOther Names:\u003c\/b\u003e ERCC6; CSB; DNA excision repair protein ERCC-6; ATP-dependent helicase ERCC6; Cockayne syndrome protein CSB\u003c\/li\u003e\n\u003cli\u003e\n\u003cb\u003eBackground:\u003c\/b\u003e 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. Alternative splicing occurs between a splice site from exon 5 of this gene to the 3' splice site upstream of the open reading frame (ORF) of the adjacent gene, piggyback-derived-3 (GeneID:267004), which activates the alternative polyadenylation site downstream of the piggyback-derived-3 ORF. The resulting transcripts encode a fusion protein that shares sequence with the product of each individual gene. [provided by RefSeq, Mar 2016],\u003c\/li\u003e\n\u003cli\u003e\n\u003cb\u003eFunction:\u003c\/b\u003e disease: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.,disease: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.,disease: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,\u003c\/li\u003e\n\u003cli\u003e\n\u003cb\u003eSpecies:\u003c\/b\u003e Human;Rat;Mouse;\u003c\/li\u003e\n\u003cli\u003e\n\u003cb\u003eRange:\u003c\/b\u003e IHC;IF;ELISA\u003c\/li\u003e\n\u003cli\u003e\n\u003cb\u003eProtein:\u003c\/b\u003e DNA excision repair protein ERCC-6\u003c\/li\u003e\n\u003c\/ul\u003e","brand":"Upingbio","offers":[{"title":"100UL","offer_id":50689879376152,"sku":"YP-Ab-01629-100UL","price":0.0,"currency_code":"USD","in_stock":true}],"thumbnail_url":"\/\/cdn.shopify.com\/s\/files\/1\/0590\/5652\/1400\/files\/images_68d688c1-71a9-421a-9e25-8b0a746296b8.png?v=1758592080","url":"https:\/\/danabiosci.com\/products\/yp-ab-01629-100ul","provider":"Dana Bioscience","version":"1.0","type":"link"}