{"product_id":"abcam-ab55080","title":"Abcam, ab55080, Anti-GBA antibody [2E2]","description":"\u003cp\u003eSize: 100µg\u003cbr\u003e\nAnti-GBA antibody [2E2] (ab55080) is a mouse monoclonal antibody detecting GBA in  Western Blot, IHC-P . Suitable for  Human . - KO validated for confirmed specificity - Over 10 publications - Trusted since 2007\u003cbr\u003e\nKey facts\u003cbr\u003e\nHost species:Mouse,\u003cbr\u003e\nClonality:Monoclonal,\u003cbr\u003e\nClone number:2E2,\u003cbr\u003e\nIsotype:IgG2a,\u003cbr\u003e\nLight chain type:kappa,\u003cbr\u003e\nCarrier free:No,\u003cbr\u003e\nReacts with:Human,\u003cbr\u003e\nApplications:IHC-P, WBSee reactivity dataSee the reactivity data table below for information on validated species and application combinations.,\u003cbr\u003e\nImmunogen:Recombinant Fragment Protein within Human GBA1 aa 100-250. The exact immunogen used to generate this antibody is proprietary information.P04062\u003c\/p\u003e\n\n\u003cp\u003eProduct details:\u003cbr\u003e\nThis product was changed from ascites to tissue culture supernatant on 15 May 2019. Please note that the dilutions may need to be adjusted accordingly. If you have any questions, please do not hesitate to contact our scientific support team.\u003cbr\u003e\nProduct Specifications\u003cbr\u003e\nAnti-GBA antibody [2E2] (ab55080) is a mouse monoclonal antibody and is validated for use in ICC\/IF, IHC-P, WB in human samples.\u003cbr\u003e\nAnti-GBA antibody [2E2] (ab55080) specifically detects GBA (UniProt ID: P04062; Molecular weight: 56kDa) and is sold in 100 µg selling sizes.\u003cbr\u003e\nQuality and Validation\u003cbr\u003e\nAbcam's high quality validation processes ensure Anti-GBA antibody [2E2] (ab55080) has high sensitivity and specificity.\u003cbr\u003e\nThe specificity of Anti-GBA antibody [2E2] (ab55080) has been confirmed by testing in knockout samples.\u003cbr\u003e\nAnti-GBA antibody [2E2] (ab55080) has been cited over 14 times in peer reviewed journals and is trusted by the scientific community.\u003cbr\u003e\nRelated Products\u003cbr\u003e\nAntibody clone 2E2 is also available pre-conjugated to a variety of labels for your convenience - PE (\u003cbr\u003e\nab95673\u003c\/p\u003e\n\n\u003cp\u003eProperties and Storage Information:\u003cbr\u003e\nForm-Liquid, Purification technique-Affinity purification Protein A, Storage buffer-pH: 7.4, Shipped at conditions-Blue Ice, 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\nGBA also known as glucosylceramidase is a lysosomal enzyme with a molecular mass of approximately 59 kDa. This enzyme breaks down glucosylceramide into glucose and ceramide. GBA is expressed predominantly in tissues with high metabolic activities such as the brain liver and spleen. Its function relies on its catalytic activity where substrates bind to its active site enabling the hydrolysis process necessary for maintaining cellular metabolism.\u003cbr\u003e\nBiological function summary\u003cbr\u003e\nGBA plays an important role in sphingolipid metabolism. It participates in the degradation of glycolipids within the lysosome contributing to lipid recycling. It acts independently rather than as a part of a major enzymatic complex. Through its role in degrading glucosylceramide GBA influences cellular homeostasis and bioenergetics ensuring balance in neural and systemic lipid levels.\u003cbr\u003e\nPathways\u003cbr\u003e\nGBA’s enzymatic functions are integral to the glycosphingolipid metabolic pathway. It is involved in the downstream steps of the lysosomal degradation of glycosphingolipids. The pathway operates alongside other important proteins such as beta-glucosidase and CERT-related transfer proteins all of which contribute to membrane lipid organization and signal transduction processes.\u003cbr\u003e\nGBA mutations are linked with Gaucher disease and Parkinson’s disease. In Gaucher disease deficient GBA activity leads to substrate accumulation resulting in hepatosplenomegaly and other systemic symptoms. Reduced GBA activity is also associated with increased alpha-synuclein aggregation in Parkinson’s disease implicating it in the pathogenesis of neurodegenerative disorders. The enzyme’s function in these diseases highlights its role in maintaining cellular equilibrium and signaling pathways.\u003c\/p\u003e","brand":"Abcam","offers":[{"title":"Default Title","offer_id":46845018079401,"sku":"ab55080","price":0.99,"currency_code":"USD","in_stock":true}],"url":"https:\/\/iright.com\/products\/abcam-ab55080","provider":"Iright","version":"1.0","type":"link"}