NDUFA1抗体—艾普蒂

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     Gel: 12%SDS-PAGE, Lysate: 40 μg, Lane 1-4: 293T cell, Mouse brain tissue, Mouse heart tissue, Rat skeletal muscle tissue lysates, Primary antibody: P08546(NDUFA1 Antibody) at dilution 1/800, Secondary antibody: Goat anti rabbit IgG at 1/5000 dilution, Exposure time: 40 seconds    

  
  

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     The image is immunohistochemistry of paraffin-embedded Human breast cancer tissue using P08546(NDUFA1 Antibody) at dilution 1/100. (Original magnification: ×200)    

  
  

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     The image is immunohistochemistry of paraffin-embedded Human gastric cancer tissue using P08546(NDUFA1 Antibody) at dilution 1/100. (Original magnification: ×200)    

  
  

NDUFA1 (NADH:ubiquinone oxidoreductase subunit A1) is a critical component of mitochondrial Complex I, the largest enzyme in the electron transport chain responsible for oxidative phosphorylation. Encoded by the nuclear gene *NDUFA1* located on the X chromosome, this 19 kDa hydrophobic protein anchors the matrix arm of Complex I, facilitating electron transfer from NADH to ubiquinone. Mutations in *NDUFA1* are linked to mitochondrial disorders, notably Leigh syndrome, a severe neurodegenerative condition characterized by developmental regression, hypotonia, and respiratory failure.

NDUFA1 antibodies are essential tools in research and diagnostics, enabling the detection and quantification of NDUFA1 protein levels. These antibodies are widely used in techniques like Western blotting, immunohistochemistry (IHC), and immunofluorescence (IF) to study Complex I assembly, mitochondrial function, and disease mechanisms. Commercially available NDUFA1 antibodies are typically raised in rabbits or mice, targeting specific epitopes within the protein’s conserved regions.

In clinical contexts, NDUFA1 antibodies aid in diagnosing mitochondrial disorders by identifying protein deficiencies in patient tissues. Research applications extend to exploring cancer metabolism, neurodegenerative diseases, and aging, where mitochondrial dysfunction is implicated. Validation of these antibodies includes testing in knockout models or siRNA-treated cells to confirm specificity. Ongoing studies also investigate NDUFA1’s role as a potential biomarker or therapeutic target for mitochondrial-related pathologies.