{"product_id":"abcam-ab258111","title":"Abcam, ab258111, Human PHKA1 knockout HEK-293T cell lysate","description":"\u003cp\u003eSize: 1Kit\u003cbr\u003e\nPHKA1 KO cell lysate available now. KO validated by Western blot. Free of charge wild type control included. Knockout achieved by using CRISPR\/Cas9, 2 bp deletion in exon14.\u003cbr\u003e\nKey facts\u003cbr\u003e\nCell type:HEK-293T,\u003cbr\u003e\nSpecies or organism:Human,\u003cbr\u003e\nTissue:Kidney,\u003cbr\u003e\nKnockout validation:Sanger Sequencing,Western blot,\u003cbr\u003e\nMutation description:Knockout achieved by using CRISPR\/Cas9, 2 bp deletion in exon14.\u003c\/p\u003e\n\n\u003cp\u003eProduct details:\u003cbr\u003e\nKnockout cell lysate achieved by CRISPR\/Cas9.\u003cbr\u003e\nREACH authorisation\u003cbr\u003e\nAbcam has not and does not intend to apply for the REACH Authorisation of customers' uses of products that contain European Authorisation list (Annex XIV) substances.\u003cbr\u003e\nIt is the responsibility of our customers to check the necessity of application of REACH Authorisation, and any other relevant authorisations, for their intended uses.\u003cbr\u003e\nLysate preparation:\u003cbr\u003e\nOur lysates are made using RIPA buffer to which we add a protease inhibitor cocktail and phosphatase inhibitor cocktail (ratio: 300:100:10).\u003cbr\u003e\nThis means that the protein of interest is denatured.\u003cbr\u003e\nIf you require a native form of the protein please use the live cell version. Please refer to our lysis protocol for further details on how our lysates are prepared.\u003cbr\u003e\nUser storage instructions:\u003cbr\u003e\nLyophilizate may be stored at 4°C. After reconstitution, store at -20°C for short-term storage or -80°C for long-term storage.\u003cbr\u003e\nThis product is subject to limited use licenses from The Broad Institute and ERS Genomics Limited, and is developed with patented technology. For full details of the limited use licenses and relevant 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-PHKA1, Gene editing type-Knockout, Gene editing method-CRISPR technology, Knockout validation-Sanger Sequencing, Western blot, Shipped at conditions-Ambient - Can Ship with Ice, Appropriate short-term storage conditions--20°C, Appropriate long-term storage conditions--20°C\u003c\/p\u003e\n\n\u003cp\u003eSupplementary Information:\u003cbr\u003e\nThis supplementary information is collated from multiple sources and compiled automatically.\u003cbr\u003e\nPHKA1 also known as phosphorylase kinase alpha 1 is a regulatory subunit of the phosphorylase kinase enzyme complex with a molecular mass around 138 kDa. This protein is mainly expressed in skeletal muscle and heart tissue. PHKA1 plays an important role in glycogen metabolism by regulating the conversion of phosphorylase b to phosphorylase a which ultimately leads to glycogen breakdown.\u003cbr\u003e\nBiological function summary\u003cbr\u003e\nGlycogen metabolism involves PHKA1 as part of the phosphorylase kinase complex. This complex exists in the muscle and liver and includes alpha beta gamma and delta subunits. PHKA1 binds together with these subunits to form a large complex helping to regulate the activity state of the enzyme. The regulation of glycogen phosphorylase is important for maintaining energy supply in cells particularly during physical activity.\u003cbr\u003e\nPathways\u003cbr\u003e\nThe activity of PHKA1 impacts glycogenolysis which is a significant metabolic pathway responsible for breaking down glycogen into glucose-1-phosphate. This pathway involves other proteins like glycogen phosphorylase and phosphoprotein phosphatase 1. PHKA1 has interactions within the cascade that ensures proper glycogen breakdown especially during conditions requiring increased energy output.\u003cbr\u003e\nMutations in PHKA1 are linked to glycogen storage disease type IXd (GSD IXd) which primarily affects muscle tissue. This condition features exercise intolerance and muscle weakness due to impaired glycogen breakdown. PHKB another homologous phosphorylase kinase subunit can connect to similar energy metabolism disruptions showing a clear association with the disorder pathway alongside PHKA1.\u003c\/p\u003e","brand":"Abcam","offers":[{"title":"Default Title","offer_id":46845169828009,"sku":"ab258111","price":0.99,"currency_code":"USD","in_stock":true}],"url":"https:\/\/iright.com\/ar\/products\/abcam-ab258111","provider":"Iright","version":"1.0","type":"link"}