EARS2抗体;EARS2 Antibody—艾普蒂

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     The image is immunohistochemistry of paraffin-embedded Human liver cancer tissue using P13711(EARS2 Antibody) at dilution 1/20. (Original magnification: ×200)    

  
  

The EARS2 (Glutamyl-tRNA Synthetase 2) antibody is a research tool targeting the mitochondrial enzyme EARS2. which plays a critical role in mitochondrial protein synthesis. EARS2 is one of several aminoacyl-tRNA synthetases (aaRSs) responsible for charging tRNA molecules with their corresponding amino acids—in this case, attaching glutamic acid to mitochondrial tRNA^(Glu). This step is essential for accurate translation of mitochondrial DNA-encoded proteins, particularly components of the electron transport chain (ETC) involved in oxidative phosphorylation.

Mutations in the EARS2 gene are linked to mitochondrial disorders, such as combined oxidative phosphorylation deficiency 12 (COXPD12), characterized by early-onset leukoencephalopathy, hypotonia, and developmental delays. Studies of EARS2 antibodies (often produced in rabbits or mice) focus on detecting protein expression, subcellular localization (via immunofluorescence), and quantification (via Western blot) in tissues or cell lines. These antibodies help elucidate EARS2's role in mitochondrial dysfunction, disease mechanisms, and potential therapeutic targets. Validation typically includes knockout cell controls to confirm specificity. Commercial EARS2 antibodies are widely used in neurology and mitochondrial research, emphasizing their importance in understanding rare genetic diseases and energy metabolism pathways.