ABCD1抗体;ABCD1 Antibody—艾普蒂

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     The image is immunohistochemistry of paraffin-embedded Human thyroid cancer tissue using P12793(ABCD1 Antibody) at dilution 1/80. (Original magnification: ×200)    

  
  

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     The image is immunohistochemistry of paraffin-embedded Human tonsil tissue using P12793(ABCD1 Antibody) at dilution 1/80. (Original magnification: ×200)    

  
  

The ABCD1 antibody targets the ABCD1 protein, a member of the ATP-binding cassette (ABC) transporter superfamily. ABCD1. also known as adrenoleukodystrophy protein (ALDP), is localized to peroxisomal membranes and plays a critical role in transporting very long-chain fatty acids (VLCFAs) into peroxisomes for β-oxidation. Mutations in the ABCD1 gene cause X-linked adrenoleukodystrophy (X-ALD), a rare neurodegenerative disorder characterized by impaired VLCFA metabolism, leading to progressive demyelination, adrenal insufficiency, and neurological decline.

ABCD1 antibodies are essential tools in research and diagnostics. They enable the detection of ABCD1 expression levels and subcellular localization via techniques like Western blotting, immunohistochemistry, and immunofluorescence. These antibodies help elucidate ABCD1's functional role in peroxisomal metabolism and its association with disease mechanisms. Clinically, they aid in confirming X-ALD diagnoses by identifying protein deficiencies or aberrant expression in patient samples. Additionally, ABCD1 antibodies support therapeutic development, including gene therapy and pharmacological approaches aimed at restoring ABCD1 function or compensating for metabolic defects. Their specificity and reliability are crucial for advancing understanding of peroxisomal disorders and improving diagnostic accuracy.