{"product_id":"abcam-ab266770","title":"Abcam, ab266770, Human PRKCSH (Glucosidase 2 subunit beta) knockout HEK-293T cell line","description":"\u003cp\u003eSize: 2 x 1000000Cells \/ vial \/ 1000000Cells \/ vial\u003cbr\u003e\nPRKCSH KO cell line available to order. KO validated by Western blot. Free of charge wild type control available. Knockout achieved by using CRISPR\/Cas9, 13 bp deletion in exon 1 and 14 bp deletion in exon 1 and Insertion of the selection cassette in exon 1. To order both knockout and wild-type control cells: select 2 x 1000000Cells\/vial. To order only knockout cells: select 1000000Cells\/vial.\u003cbr\u003e\nKey facts\u003cbr\u003e\nCell type:HEK-293T,\u003cbr\u003e\nSpecies or organism:Human,\u003cbr\u003e\nTissue:Kidney,\u003cbr\u003e\nForm:LiquidSee storage information,\u003cbr\u003e\nKnockout validation:Sanger Sequencing,Western blot,\u003cbr\u003e\nMutation description:Knockout achieved by using CRISPR\/Cas9, 13 bp deletion in exon 1 and 14 bp deletion in exon 1 and Insertion of the selection cassette in exon 1\u003c\/p\u003e\n\n\u003cp\u003eProduct details:\u003cbr\u003e\nWe will provide viable cells that proliferate on revival.\u003cbr\u003e\nThis product is subject to limited use licenses from The Broad Institute, ERS Genomics Limited and Sigma-Aldrich Co. LLC, and is developed with patented technology. For full details of the licenses and patents please refer to our\u003cbr\u003e\nlimited use license\u003cbr\u003e\npatent pages\u003c\/p\u003e\n\n\u003cp\u003eProperties and Storage Information:\u003cbr\u003e\nGene name-PRKCSH, Gene editing type-Knockout, Gene editing method-CRISPR technology, Knockout validation-Sanger Sequencing, Western blot, Shipped at conditions-Dry Ice, Appropriate short-term storage conditions--196°C, Appropriate long-term storage conditions--196°C\u003c\/p\u003e\n\n\u003cp\u003eSupplementary Information:\u003cbr\u003e\nThis supplementary information is collated from multiple sources and compiled automatically.\u003cbr\u003e\nGlucosidase 2 subunit beta also known as PRKCSH plays an important role in protein processing within the endoplasmic reticulum (ER). This protein forms part of the heterodimeric enzyme complex glucosidase II responsible for cleaving glucose molecules during glycoprotein maturation. With a molecular weight of about 60 kDa glucosidase 2 subunit beta is important for efficient N-linked glycosylation which is a fundamental process for protein folding and quality control. This protein localizes mainly to the ER where it contributes to proper cellular functions by aiding in the preparation of proteins for trafficking to their final destinations.\u003cbr\u003e\nBiological function summary\u003cbr\u003e\nGlucosidase 2 subunit beta is associated with protein maturation and quality control within the ER lumen. It is part of the glucosidase II complex which functions to trim glucose residues from N-linked oligosaccharides on nascent glycoproteins facilitating their proper folding and preventing misfolded proteins' accumulation. This activity ensures that only properly folded proteins progress through the secretory pathway while faulty proteins are targeted for degradation. Through these processes glucosidase 2 subunit beta supports cellular homeostasis and protein turnover.\u003cbr\u003e\nPathways\u003cbr\u003e\nGlucosidase 2 subunit beta plays an influential role in the protein processing and ER-associated degradation (ERAD) pathways. The protein operates within these pathways to regulate glycoprotein folding impacting the unfolding protein response (UPR). It interacts with several proteins including calnexin and calreticulin which are key players in the glycoprotein folding process. Moreover the proper function of glucosidase 2 subunit beta in these pathways is necessary for maintaining the cellular stress response ultimately ensuring efficient cell function and survival.\u003cbr\u003e\nDefects in glucosidase 2 subunit beta have links to autosomal dominant polycystic liver disease (PCLD). Mutations in PRKCSH which encodes glucosidase 2 subunit beta can lead to the development of PCLD due to disrupted glycoprotein folding and ER stress-induced apoptosis. Additionally abnormalities in its function may indirectly connect to diabetes mellitus by affecting insulin receptor glycosylation and secretion processes. These relationships illustrate the broader impact of glucosidase 2 subunit beta on health and disease providing insight into potential therapeutic targets for treatment strategies.\u003c\/p\u003e","brand":"Abcam","offers":[{"title":"Default Title","offer_id":46845066641577,"sku":"ab266770","price":0.99,"currency_code":"USD","in_stock":true}],"url":"https:\/\/iright.com\/ar\/products\/abcam-ab266770","provider":"Iright","version":"1.0","type":"link"}