FAH抗体—艾普蒂

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     Gel: 8%SDS-PAGE, Lysate: 40 μg, Lane: Human liver tissue lysate, Primary antibody: P02714(FAH Antibody) at dilution 1/700, Secondary antibody: Goat anti rabbit IgG at 1/8000 dilution, Exposure time: 30 seconds    

  
  

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     The image is immunohistochemistry of paraffin-embedded Human ovarian cancer tissue using P02714(FAH Antibody) at dilution 1/40. (Original magnification: ×200)    

  
  

Fumarylacetoacetate hydrolase (FAH) is a critical enzyme in the tyrosine catabolic pathway, catalyzing the final step that converts fumarylacetoacetate into fumarate, acetoacetate, and water. FAH deficiency causes hereditary tyrosinemia type 1 (HT1), a rare autosomal recessive disorder characterized by severe liver dysfunction, renal tubular damage, and neurological crises. FAH antibodies are immunological tools developed to detect and quantify FAH protein levels in research and diagnostic settings. These antibodies are essential for studying FAH expression patterns, understanding disease mechanisms in HT1. and evaluating experimental therapies, such as gene correction or enzyme replacement strategies.

In research, FAH antibodies are widely used in techniques like Western blotting, immunohistochemistry, and immunofluorescence to localize FAH in tissues (primarily liver and kidney) and assess its expression under pathological or experimental conditions. They also aid in creating FAH-deficient animal models (e.g., mice) to mimic HT1 for therapeutic testing. Commercially available FAH antibodies are typically raised in rabbits or mice using peptide antigens derived from conserved regions of the human FAH protein. Specificity and validation data (e.g., knockout-validated) are crucial for ensuring reliable results. Beyond HT1. FAH antibodies have exploratory roles in cancer studies, as tyrosine metabolism dysregulation is linked to tumor progression. Overall, FAH antibodies serve as vital reagents bridging basic research, diagnostics, and translational medicine.