{"product_id":"abcam-ab266419","title":"Abcam, ab266419, Human NDUFS3 knockout HEK-293T cell line","description":"\u003cp\u003eSize: 2 x 1000000Cells \/ vial \/ 1000000Cells \/ vial\u003cbr\u003e\nNDUFS3 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: 19 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:HEK-293T,\u003cbr\u003e\nSpecies or organism:Human,\u003cbr\u003e\nTissue:Kidney,\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: 19 bp deletion in exon 1\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-NDUFS3, 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\nNADH:ubiquinone oxidoreductase core subunit S3 (NDUFS3) is an important component of the mitochondrial respiratory chain complex I also known as NADH:ubiquinone oxidoreductase. With an approximate mass of 25 kDa NDUFS3 plays an integral role in the assembly and function of complex I. This protein is mostly expressed in the mitochondria across various tissues. It is better known as a core subunit important for the catalytic activity of complex I.\u003cbr\u003e\nBiological function summary\u003cbr\u003e\nNDUFS3 participates in cellular respiration by facilitating the electron transfer from NADH to ubiquinone. It is part of the complex I assembly that comprises 45 different subunits. This large assembly is the first enzyme of the mitochondrial electron transport chain and it ensures efficient energy production in the form of ATP. In its role NDUFS3 collaborates closely with other core subunits like NDUFS1 and NDUFS2 to maintain the proper function of cellular metabolism.\u003cbr\u003e\nPathways\u003cbr\u003e\nNDUFS3 is essential in the oxidative phosphorylation pathway which plays an important part in ATP generation. By interacting with other complex I subunits NDUFS3 enables electron flow that drives ATP synthase activity. Additionally it is connected to the apoptosis pathway. Its dysfunction may result in disrupted energy metabolism which can trigger cell death. NDUFS3 also interacts with proteins like NDUFV1 and NDUFV2 highlighting its centrality in energy and signal transduction processes.\u003cbr\u003e\nNDUFS3 links to mitochondrial disorders and neurodegenerative diseases such as Leigh syndrome. Mutations in NDUFS3 can impair mitochondrial function leading to reduced ATP production and accumulation of defective mitochondria. These alterations contribute to the progressive deterioration seen in these disorders. In the context of neurodegenerative diseases NDUFS3 dysfunction may associate with proteins like cytochrome c emphasizing its role in mitochondria-dependent apoptotic pathways and neuronal health.\u003c\/p\u003e","brand":"Abcam","offers":[{"title":"Default Title","offer_id":46845079027881,"sku":"ab266419","price":0.99,"currency_code":"USD","in_stock":true}],"url":"https:\/\/iright.com\/es\/products\/abcam-ab266419","provider":"Iright","version":"1.0","type":"link"}