{"product_id":"abcam-ab275596","title":"Abcam, ab275596, Anti-ERCC8 antibody","description":"\u003cp\u003eSize: 100µL\u003cbr\u003e\nRabbit Polyclonal ERCC8 antibody. Suitable for WB, ICC\/IF and reacts with Mouse, Human samples. Immunogen corresponding to Synthetic Peptide within Human ERCC8.\u003cbr\u003e\nKey facts\u003cbr\u003e\nHost species:Rabbit,\u003cbr\u003e\nClonality:Polyclonal,\u003cbr\u003e\nIsotype:IgG,\u003cbr\u003e\nCarrier free:No,\u003cbr\u003e\nReacts with:Mouse, Human,\u003cbr\u003e\nApplications:WB, ICC\/IFSee reactivity dataSee the reactivity data table below for information on validated species and application combinations.,\u003cbr\u003e\nImmunogen:Synthetic Peptide within Human ERCC8. The exact immunogen used to generate this antibody is proprietary information.Q13216\u003c\/p\u003e\n\n\u003cp\u003eProperties and Storage Information:\u003cbr\u003e\nForm-Liquid, Purification technique-Affinity purification Protein A, Storage buffer-Constituents: PBS, Shipped at conditions-Blue Ice, Appropriate short-term storage duration-1-2 weeks, Appropriate short-term storage conditions-+4°C, Appropriate long-term storage conditions--20°C, Aliquoting information-Upon delivery aliquot, Storage information-Avoid freeze \/ thaw cycle\u003c\/p\u003e\n\n\u003cp\u003eSupplementary Information:\u003cbr\u003e\nThis supplementary information is collated from multiple sources and compiled automatically.\u003cbr\u003e\nERCC8 also known as CSA protein plays an essential role in the repair of transcription-coupled nucleotide excision repair (TC-NER) pathways. This protein has a mass of about 44 kDa. Expression of ERCC8 is found in several tissues notably in the skin liver and nervous system. It helps in recognizing and repairing DNA damage that occurs during transcription.\u003cbr\u003e\nBiological function summary\u003cbr\u003e\nERCC8 forms a part of the CSA complex which includes other proteins involved in the TC-NER pathway. The CSA complex targets the repair machinery to the site of transcription-blocking lesions. Identifying these lesions is critical to resuming normal transcription processes. The CSA protein therefore actively contributes to maintaining genomic stability by facilitating the correction of transcription-coupled DNA damage.\u003cbr\u003e\nPathways\u003cbr\u003e\nERCC8 participates directly in the nucleotide excision repair (NER) pathway specifically in its transcription-coupled repair subprocess. This pathway is integral to correcting helix-distorting DNA lesions that impede transcription. ERCC8 interacts closely with the Cockayne syndrome B (CSB) protein and RNA polymerase II during the repair process. This collaboration is important in modulating the restart of transcription post-repair.\u003cbr\u003e\nMutations in ERCC8 lead to Cockayne syndrome (CS) characterized by growth defects neurological dysfunction and photosensitivity. ERCC8 alterations are linked primarily to Cockayne syndrome group A. The protein is also involved in repair mechanisms tied to UV-sensitive syndrome. In both conditions faulty ERCC8 function can disrupt normal DNA repair processes leading to severe clinical manifestations associated with DNA repair failure. The role of CSA alongside CSB mutations further highlights its importance in disease phenotypes and the need for effective DNA repair.\u003c\/p\u003e","brand":"Abcam","offers":[{"title":"Default Title","offer_id":46854233751721,"sku":"ab275596","price":0.99,"currency_code":"USD","in_stock":true}],"url":"https:\/\/iright.com\/products\/abcam-ab275596","provider":"Iright","version":"1.0","type":"link"}