ADAMTS13 autoantibody-expressing B cell receptor (ADAMTS13-specific BCR)

The ADAMTS13 autoantibody-expressing B cell receptor is a membrane-bound immunoglobulin found on the surface of B lymphocytes that specifically recognizes the ADAMTS13 metalloprotease. In patients with acquired thrombotic thrombocytopenic purpura (aTTP), these B cells differentiate into plasma cells that secrete high-affinity autoantibodies against ADAMTS13, leading to its functional deficiency (https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5543935/). This deficiency results in the accumulation of ultra-large von Willebrand factor multimers, which cause microvascular thrombosis and systemic complications (https://pubmed.ncbi.nlm.nih.gov/28438767/). Therapeutic strategies targeting these specific receptors, such as Chimeric Autoantibody Receptor (CAAR) T-cell therapy, aim to selectively deplete the pathogenic B-cell population while sparing healthy B cells (https://ashpublications.org/blood/article/132/11/1103/39453/B-cell-receptors-in-acquired-TTP). This approach offers a more precise alternative to broad immunosuppressants like rituximab, potentially reducing the risk of long-term immunodeficiency and improving patient outcomes in refractory cases.

Other names

Anti-ADAMTS13 B-cell receptorADAMTS13-specific B-cell receptoraTTP-associated B-cell receptorADAMTS13-reactive B-cell receptor

Selective or broad depletion of B-cell populations expressing the ADAMTS13-specific receptor to halt the production of pathogenic autoantibodies and restore ADAMTS13-mediated cleavage of von Willebrand factor (https://onlinelibrary.wiley.com/doi/full/10.1111/jth.14745).