Description
Key facts
Cell type:HEK-293T,
Species or organism:Human,
Tissue:Kidney,
Knockout validation:Sanger Sequencing,Western blot,
Mutation description:Knockout achieved by using CRISPR/Cas9, Homozygous: 19 bp deletion in exon 1.
Product details:
Knockout cell lysate achieved by CRISPR/Cas9.
REACH authorisation
Abcam has not and does not intend to apply for the REACH Authorisation of customers' uses of products that contain European Authorisation list (Annex XIV) substances.
It is the responsibility of our customers to check the necessity of application of REACH Authorisation, and any other relevant authorisations, for their intended uses.
Lysate preparation:
Our lysates are made using RIPA buffer to which we add a protease inhibitor cocktail and phosphatase inhibitor cocktail (ratio: 300:100:10).
This means that the protein of interest is denatured.
If you require a native form of the protein please use the live cell version. Please refer to our lysis protocol for further details on how our lysates are prepared.
User storage instructions:
Lyophilizate may be stored at 4°C. After reconstitution, store at -20°C for short-term storage or -80°C for long-term storage.
This 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
limited use license
patent pages
Properties and Storage Information:
Gene name-NDUFS3, Gene editing type-Knockout, Gene editing method-CRISPR technology, Knockout validation-Sanger Sequencing, Western blot, Zygosity-Homozygous, Shipped at conditions-Ambient - Can Ship with Ice, Appropriate short-term storage conditions--20°C, Appropriate long-term storage conditions--20°C
Supplementary Information:
This supplementary information is collated from multiple sources and compiled automatically.
NADH: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.
Biological function summary
NDUFS3 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.
Pathways
NDUFS3 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.
NDUFS3 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.
