{"product_id":"abcam-ab248929","title":"Abcam, ab248929, Anti-HEXB antibody [EPR7978] - BSA and Azide free","description":"\u003cp\u003eSize: 100µg \/ 1mg\u003cbr\u003e\nRabbit Recombinant Monoclonal HEXB antibody. Carrier free. Suitable for WB, IHC-P and reacts with Human samples. Cited in 1 publication.\u003cbr\u003e\nKey facts\u003cbr\u003e\nHost species:Rabbit,\u003cbr\u003e\nClonality:Monoclonal,\u003cbr\u003e\nClone number:EPR7978,\u003cbr\u003e\nIsotype:IgG,\u003cbr\u003e\nCarrier free:Yes,\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:The exact immunogen used to generate this antibody is proprietary information.\u003c\/p\u003e\n\n\u003cp\u003eProduct details:\u003cbr\u003e\nab248929 is the carrier-free version of\u003cbr\u003e\nab140649\u003cbr\u003e\nPatented technology\u003cbr\u003e\nOur RabMAb\u003cbr\u003e\ntechnology is a patented hybridoma-based technology for making rabbit monoclonal antibodies. For details on our patents, please refer to\u003cbr\u003e\nRabMAb® patents\u003cbr\u003e\nWhat are the advantages of a recombinant monoclonal antibody?\u003cbr\u003e\nThis product is a recombinant monoclonal antibody, which offers several advantages including:\u003cbr\u003e\n- High batch-to-batch consistency and reproducibility\u003cbr\u003e\n- Improved sensitivity and specificity\u003cbr\u003e\n- Long-term security of supply\u003cbr\u003e\n- Animal-free batch production\u003cbr\u003e\nFor more information, read more on\u003cbr\u003e\nrecombinant antibodies\u003cbr\u003e\nConjugation ready\u003cbr\u003e\nOur carrier-free antibodies are typically supplied in a PBS-only formulation, purified and free of BSA, sodium azide and glycerol. This conjugation-ready format is designed for use with fluorochromes, metal isotopes, oligonucleotides, and enzymes, which makes them ideal for antibody labelling, functional and cell-based assays, flow-based assays (e.g. mass cytometry) and Multiplex Imaging applications.\u003cbr\u003e\nUse our\u003cbr\u003e\nconjugation kits\u003cbr\u003e\nfor antibody conjugates that are ready-to-use in as little as 20 minutes with 1 minute hands-on-time and 100% antibody recovery: available for fluorescent dyes, HRP, biotin and gold.\u003cbr\u003e\nCompatibility\u003cbr\u003e\nThis product is compatible with the Maxpar\u003cbr\u003e\nAntibody Labeling Kit from Fluidigm, without the need for antibody preparation. Maxpar\u003cbr\u003e\nis a trademark of Fluidigm Canada Inc.\u003c\/p\u003e\n\n\u003cp\u003eProperties and Storage Information:\u003cbr\u003e\nForm-Liquid, Purification technique-Affinity purification Protein A, Storage buffer-pH: 7.2 - 7.4Constituents: PBS, Shipped at conditions-Blue Ice, Appropriate short-term storage conditions-+4°C, Appropriate long-term storage conditions-+4°C, Storage information-Do Not Freeze\u003c\/p\u003e\n\n\u003cp\u003eSupplementary Information:\u003cbr\u003e\nThis supplementary information is collated from multiple sources and compiled automatically.\u003cbr\u003e\nThe target known as HEXB also called Hexosaminidase subunit beta consists of protein subunit that helps in the formation of the enzyme beta-hexosaminidase. This enzyme is active in lysosomes and it weighs about 63 kDa. Beta-hexosaminidase functions to break down GM2 gangliosides which are molecules in cell membranes. HEXB expresses mainly in the brain and liver tissues signaling its role in neurological and hepatic processes.\u003cbr\u003e\nBiological function summary\u003cbr\u003e\nThe beta-hexosaminidase enzyme formed by HEXB participates in the lysosomal degradation pathway. This enzyme requires formation of a complex with its counterpart HEXA to become biologically active. Together they catalyze the hydrolysis of GM2 gangliosides into GM3 gangliosides which is an essential step for cell membrane metabolism. This process is important for maintaining cellular homeostasis and preventing accumulation of toxic substances within cells.\u003cbr\u003e\nPathways\u003cbr\u003e\nThe HEXB encoded enzyme participates in sphingolipid metabolism an integral component of lipid metabolic pathways. Sphingolipids are part of many cellular functions including signal transmission and cell recognition. HEXB through the lysosomal degradation of gangliosides works closely with HEXA and NEU1 which further processes GM3 gangliosides into simpler molecules. Such interactions underline HEXB’s significant role in maintaining the balance of lipid metabolism within cells.\u003cbr\u003e\nMutations or deficiencies in HEXB lead to serious genetic conditions such as Sandhoff disease. This disorder results from the improper breakdown of gangliosides due to the lack of functional HEXB leading to their accumulation which severely damages nerve cells and results in neurological impairments. The partnership between HEXB and HEXA emphasizes the importance of both subunits as malfunction in either can contribute to disease progression by disrupting normal ganglioside metabolism.\u003c\/p\u003e","brand":"Abcam","offers":[{"title":"Default Title","offer_id":46851789750441,"sku":"ab248929","price":0.99,"currency_code":"USD","in_stock":true}],"url":"https:\/\/iright.com\/ar\/products\/abcam-ab248929","provider":"Iright","version":"1.0","type":"link"}