OTC抗体—艾普蒂

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     Gel: 8%SDS-PAGE, Lysate: 40 μg, Lane: Mouse liver tissue, Primary antibody: P08589(OTC Antibody) at dilution 1/650, Secondary antibody: Goat anti rabbit IgG at 1/8000 dilution, Exposure time: 1 second    

  
  

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     The image is immunohistochemistry of paraffin-embedded Human colon cancer tissue using P08589(OTC Antibody) at dilution 1/40. (Original magnification: ×200)    

  
  

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     The image is immunohistochemistry of paraffin-embedded Human tonsil tissue using P08589(OTC Antibody) at dilution 1/40. (Original magnification: ×200)    

  
  

OTC antibodies target ornithine transcarbamylase (OTC), a mitochondrial enzyme critical to the urea cycle, which detoxifies ammonia by converting it into urea. As the second enzymatic step in this cycle, OTC catalyzes the reaction between ornithine and carbamoyl phosphate to form citrulline. Inherited deficiencies in OTC, an X-linked disorder, are the most common urea cycle defect, causing hyperammonemia with severe neurological or fatal outcomes if untreated.

OTC antibodies are vital tools in diagnosing and researching this condition. They enable detection of OTC protein levels via techniques like Western blot, immunohistochemistry, or ELISA, helping confirm genetic diagnoses, assess residual enzyme activity, and study tissue-specific expression. In research, these antibodies aid in exploring urea cycle regulation, mitochondrial metabolism, and disease mechanisms. They also support preclinical studies for gene therapies or enzyme replacement strategies.

Commercial OTC antibodies are typically raised in rabbits or mice using recombinant human OTC protein epitopes. Specificity validation (e.g., knockout controls) is essential due to potential cross-reactivity with related proteins. Beyond diagnostics, they contribute to understanding metabolic adaptations in liver diseases, cancer, or drug-induced mitochondrial dysfunction, highlighting OTC's broader pathophysiological roles.