{"product_id":"abcam-ab300448","title":"Abcam, ab300448, Anti-HEXA antibody [EPR26394-74] (BSA and Azide free)","description":"\u003cp\u003eSize: 100µg \/ 1mg\u003cbr\u003e\nRabbit Recombinant Monoclonal HEXA antibody. Carrier free. Suitable for IP, Flow Cyt (Intra), ICC\/IF, IHC-P, IHC-Fr, WB and reacts with Mouse, Rat samples.\u003cbr\u003e\nKey facts\u003cbr\u003e\nHost species:Rabbit,\u003cbr\u003e\nClonality:Monoclonal,\u003cbr\u003e\nClone number:EPR26394-74,\u003cbr\u003e\nIsotype:IgG,\u003cbr\u003e\nCarrier free:Yes,\u003cbr\u003e\nReacts with:Rat, Mouse,\u003cbr\u003e\nApplications:IP, IHC-Fr, Flow Cyt (Intra), WB, ICC\/IF, IHC-PSee 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\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 HEXA gene codes for the enzyme Hexosaminidase A also known as hexosaminidase alpha or HEXA subunit. This enzyme has several subunits with a molecular mass of approximately 58 kDa. HEXA expresses in various tissues but it shows high expression in the brain and other neural tissues. Mechanically Hexosaminidase A is involved in the hydrolysis of GM2 gangliosides into GM3 by removing the N-acetylgalactosamine residue which plays a role in the degradation of glycolipids within lysosomes.\u003cbr\u003e\nBiological function summary\u003cbr\u003e\nHexosaminidase A functions as part of the lysosomal enzyme complex partnering with the GM2 activator protein and another related enzyme Hexosaminidase B. The complex is critical in the catabolism of GM2 gangliosides a type of lipid found in cell membranes specifically in neuronal cell membranes. Efficient function of Hexosaminidase A prevents accumulation of the lipid ensuring cellular health particularly in neurons.\u003cbr\u003e\nPathways\u003cbr\u003e\nHexosaminidase A is significant within glycolipid metabolism pathways and lysosomal degradation pathways. It cooperates closely with enzymes like Hexosaminidase B and the GM2 activator protein within these processes. These pathways are important for the breakdown of gangliosides which prevents their accumulation and maintains cellular function in neuronal tissues.\u003cbr\u003e\nHEXA mutations have a direct link to Tay-Sachs disease a severe genetic disorder affecting nerve cells. This disorder results from HEXA mutations causing deficient enzyme activity leading to GM2 ganglioside accumulation. Additionally Sandhoff disease links through similar pathways though its relation more involves Hexosaminidase B deficiencies. Both disorders highlight the importance of a functioning Hexosaminidase A enzyme for normal neurological function.\u003c\/p\u003e","brand":"Abcam","offers":[{"title":"Default Title","offer_id":46851619291305,"sku":"ab300448","price":0.99,"currency_code":"USD","in_stock":true}],"url":"https:\/\/iright.com\/products\/abcam-ab300448","provider":"Iright","version":"1.0","type":"link"}