{"product_id":"abcam-ab230488","title":"Abcam, ab230488, Anti-HEXA antibody","description":"\u003cp\u003eSize: 200µL\u003cbr\u003e\nRabbit Polyclonal HEXA antibody. Suitable for IHC-P, WB, Flow Cyt (Intra) and reacts with Human, Mouse samples. Cited in 2 publications. Immunogen corresponding to Synthetic Peptide within Human HEXA aa 100-200 conjugated to Keyhole Limpet Haemocyanin.\u003cbr\u003e\nKey facts\u003cbr\u003e\nHost species:Rabbit,\u003cbr\u003e\nClonality:Polyclonal,\u003cbr\u003e\nIsotype:IgG,\u003cbr\u003e\nCarrier free:No,\u003cbr\u003e\nReacts with:Mouse, Human,\u003cbr\u003e\nApplications:Flow Cyt (Intra), WB, IHC-PSee reactivity dataSee the reactivity data table below for information on validated species and application combinations.,\u003cbr\u003e\nImmunogen:Synthetic Peptide within Human HEXA aa 100-200 conjugated to Keyhole Limpet Haemocyanin. The exact immunogen used to generate this antibody is proprietary information.P06865\u003c\/p\u003e\n\n\u003cp\u003eProperties and Storage Information:\u003cbr\u003e\nForm-Liquid, Purification technique-Affinity purification Protein A, Storage buffer-pH: 7.4Preservative: 0.09% Sodium azideConstituents: PBS, Shipped at conditions-Blue Ice, Appropriate short-term storage duration-1-2 weeks, 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\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":46854034653353,"sku":"ab230488","price":0.99,"currency_code":"USD","in_stock":true}],"url":"https:\/\/iright.com\/products\/abcam-ab230488","provider":"Iright","version":"1.0","type":"link"}