ABCB4抗体;ABCB4 Antibody—艾普蒂 新品

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     The image is immunohistochemistry of paraffin-embedded Human colorectal cancer tissue using P12593(ABCB4 Antibody) at dilution 1/70. (Original magnification: ×200)    

  
  

ABCB4. a member of the ATP-binding cassette (ABC) transporter family, is encoded by the *ABCB4* gene and plays a critical role in hepatobiliary function. Expressed predominantly in the liver, it mediates the translocation of phosphatidylcholine (a major biliary phospholipid) across the canalicular membrane into bile, a process essential for protecting cholangiocytes from bile acid toxicity and maintaining bile homeostasis. Dysfunctional ABCB4 is linked to multiple cholestatic liver diseases, including progressive familial intrahepatic cholestasis type 3 (PFIC3), intrahepatic cholestasis of pregnancy (ICP), and low phospholipid-associated cholelithiasis (LPAC). These conditions often arise from mutations or reduced expression of ABCB4. leading to impaired phospholipid secretion, bile duct damage, and fibrosis.

ABCB4 antibodies are vital reagents for detecting and characterizing this transporter in research and diagnostics. They enable the visualization of ABCB4 expression patterns via techniques like Western blotting, immunohistochemistry, and immunofluorescence, aiding studies on its regulation, interaction partners, and disease-associated alterations. Clinically, these antibodies support genetic testing by confirming protein deficiency in liver biopsies, guiding diagnosis of inherited cholestatic syndromes. Furthermore, they are instrumental in developing therapeutic strategies, such as gene therapy or pharmacological chaperones, to restore ABCB4 function. By elucidating ABCB4’s role in bile formation and pathology, these antibodies contribute to advancing precision medicine for cholestatic disorders.