ETHE1抗体

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     Gel: 10%SDS-PAGE, Lysate: 40 μg, Lane 1-2: A549 cells, mouse liver tissue, Primary antibody: P08296(ETHE1 Antibody) at dilution 1/200, Secondary antibody: Goat anti rabbit IgG at 1/8000 dilution, Exposure time: 40 seconds    

  
  

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     The image is immunohistochemistry of paraffin-embedded Human thyroid cancer tissue using P08296(ETHE1 Antibody) at dilution 1/20. (Original magnification: ×200)    

  
  

The ETHE1 antibody is designed to target the ETHE1 protein, a mitochondrial enzyme encoded by the *ETHE1* gene in humans. ETHE1. also known as persulfide dioxygenase, plays a critical role in sulfur metabolism, particularly in the detoxification of hydrogen sulfide (H₂S) within mitochondria. It catalyzes the oxidation of glutathione persulfide (GSSH) to sulfite, a step essential for maintaining cellular redox balance and preventing the accumulation of toxic sulfides. Mutations in the *ETHE1* gene are linked to ethylmalonic encephalopathy (EE), a rare autosomal recessive disorder characterized by severe neurological deterioration, chronic diarrhea, and vasculopathy. EE patients exhibit disrupted sulfide metabolism, leading to elevated levels of H₂S and organic acids, which contribute to mitochondrial dysfunction and tissue damage.

The ETHE1 antibody is widely utilized in research to study the expression, localization, and functional roles of the ETHE1 protein in cellular and disease contexts. It serves as a key tool in Western blotting, immunohistochemistry, and immunofluorescence to assess protein levels in mitochondrial extracts or tissue samples. Researchers also employ this antibody to investigate pathogenic mechanisms underlying EE and to validate experimental models, such as knockout cell lines or animal studies. Additionally, it aids in diagnostic workflows to confirm ETHE1 deficiency in clinical settings. By enabling precise detection of ETHE1. this antibody supports advances in understanding mitochondrial disorders and developing potential therapeutic strategies.