IDS抗体—艾普蒂

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     The image is immunohistochemistry of paraffin-embedded Human liver cancer tissue using P10366(IDS Antibody) at dilution 1/60. (Original magnification: ×200)    

  
  

Iduronate 2-sulfatase (IDS) is a lysosomal enzyme critical for glycosaminoglycan (GAG) metabolism, specifically catalyzing the hydrolysis of sulfate groups from dermatan and heparan sulfate. Mutations in the *IDS* gene cause a deficiency in IDS activity, leading to lysosomal accumulation of GAGs and the development of mucopolysaccharidosis type II (MPS II or Hunter syndrome), a rare X-linked recessive disorder. Symptoms include skeletal deformities, organomegaly, neurological decline, and shortened lifespan.

Anti-IDS antibodies are primarily studied in the context of enzyme replacement therapy (ERT), a standard treatment for MPS II. Recombinant IDS (e.g., idursulfase) is administered to restore enzymatic activity, but some patients develop neutralizing antibodies against the therapeutic enzyme, reducing efficacy. Antibody formation correlates with disease severity, as patients with null mutations are more prone to immune responses. Monitoring anti-IDS antibody levels helps tailor treatment strategies, including dose adjustments or immunomodulation.

Additionally, anti-IDS antibodies serve as diagnostic tools to quantify endogenous IDS protein in suspected cases or carrier testing. Research also explores their role in gene therapy, where immune responses to viral vectors or expressed IDS may impact outcomes. Understanding antibody dynamics in MPS II remains vital for optimizing therapeutic interventions and improving patient prognosis.