{"product_id":"abcam-ab265563","title":"Abcam, ab265563, Human GLA (Galactosidase alpha) knockout HeLa cell line","description":"\u003cp\u003eSize: 1000000Cells \/ vial \/ 2 x 1000000Cells \/ vial\u003cbr\u003e\nGLA KO cell line available to order. KO validated by Western blot. Free of charge wild type control available. Knockout achieved by using CRISPR\/Cas9, Homozygous: 1 bp deletion in exon 1. To order both knockout and wild-type control cells: select 2 x 1000000Cells\/vial. To order only knockout cells: select 1000000Cells\/vial.\u003cbr\u003e\nKey facts\u003cbr\u003e\nCell type:HeLa,\u003cbr\u003e\nSpecies or organism:Human,\u003cbr\u003e\nTissue:Cervix,\u003cbr\u003e\nForm:LiquidSee storage information,\u003cbr\u003e\nKnockout validation:Sanger Sequencing,Western blot,\u003cbr\u003e\nMutation description:Knockout achieved by using CRISPR\/Cas9, Homozygous: 1 bp deletion in exon 1,\u003cbr\u003e\nAntibiotic resistance:Puromycin 1µg\/mL,\u003cbr\u003e\nDisease:Adenocarcinoma\u003c\/p\u003e\n\n\u003cp\u003eProduct details:\u003cbr\u003e\nWe will provide viable cells that proliferate on revival.\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-GLA, Gene editing type-Knockout, Gene editing method-CRISPR technology, Knockout validation-Sanger Sequencing, Western blot, Zygosity-Homozygous, Shipped at conditions-Dry Ice, Appropriate short-term storage conditions--196°C, Appropriate long-term storage conditions--196°C\u003c\/p\u003e\n\n\u003cp\u003eSupplementary Information:\u003cbr\u003e\nThis supplementary information is collated from multiple sources and compiled automatically.\u003cbr\u003e\nAlpha-galactosidase also known as alpha-D-galactosidase or A-galactosidase is an enzyme that catalyzes the hydrolysis of terminal alpha-galactosyl moieties from glycolipids and glycoproteins. It has a molecular mass of approximately 50000 Daltons. This enzyme is expressed in various tissues including the liver heart and kidneys. It functions by efficiently removing alpha-galactose residues from target molecules a process important for maintaining cellular function.\u003cbr\u003e\nBiological function summary\u003cbr\u003e\nThe role of alpha-galactosidase involves the breakdown of complex carbohydrates particularly those containing alpha-galactosidic bonds. It does not operate as part of a complex but rather functions independently to cleave these specific bonds in glycolipids and glycoproteins. This activity is critical for degrading langerin and other gangliosides preventing the accumulation of these molecules in the lysosomes. By continuously performing this function alpha-galactosidase maintains glycosphingolipid homeostasis ensuring efficient cellular metabolism.\u003cbr\u003e\nPathways\u003cbr\u003e\nThe involvement of alpha-galactosidase in metabolic and lysosomal catabolic pathways is significant. One critical pathway is the glycolipid metabolism pathway where it acts alongside related enzymes such as beta-galactosidase. It also participates in the lysosomal degradation pathway working in conjunction with other lysosomal enzymes to prevent substrate buildup. The interaction with enzymes like lysosomal hydrolases ensures the proper breakdown of complex molecules into simpler ones that cells can utilize or excrete.\u003cbr\u003e\nDefects in the alpha-galactosidase enzyme lead to the lysosomal storage disorder known as Fabry disease. This genetic disorder causes the accumulation of globotriaosylceramide due to deficient alpha-galactosidase activity. Another disorder associated with malfunctioning alpha-galactosidase is cardiac complications due to tissue storage of glycolipids. In both cases the lack of enzyme activity disrupts cellular and tissue functions linking alpha-galactosidase to clinical symptoms seen in these disorders.\u003c\/p\u003e","brand":"Abcam","offers":[{"title":"Default Title","offer_id":46845106421929,"sku":"ab265563","price":0.99,"currency_code":"USD","in_stock":true}],"url":"https:\/\/iright.com\/es\/products\/abcam-ab265563","provider":"Iright","version":"1.0","type":"link"}