Target intelligence / Profile preview

Autoreactive B cell receptor specific for Platelet glycoprotein Ib alpha chain (null)

Target
null
Molecular classification
Receptor, Immunoglobulin superfamily, B cell receptor (BCR), Other (Autoreactive, meaning immune tolerance/break)
01

Overview

Autoreactive B cell receptors specific for glycoprotein Ib alpha are immunoglobulin receptors expressed on B lymphocytes that mistakenly recognize platelet glycoprotein Ib alpha as an antigen. This aberrant immune recognition leads to the production of pathogenic anti-GPIbα autoantibodies, a central mechanism in certain autoimmune platelet disorders, most notably immune thrombocytopenia (ITP), resulting in platelet clearance and increased risk of bleeding. Such B cell clones are considered promising therapeutic targets for immune cell–directed therapies, including chimeric autoantibody receptor (CAAR) T cells engineered to selectively eradicate these autoreactive B cells without broadly depleting healthy B cells. Autoantibodies from these B cells not only mediate platelet destruction but also correlate with disease severity and resistance to conventional immunosuppressive treatments.

Other names
Autoreactive BCR specific for GP IbαAnti-GPIbα B cell receptorAnti–platelet glycoprotein Ib alpha B cell receptorAnti-CD42b B cell receptor
02

Mechanism of action

Elimination of autoreactive B cells by CAAR T cells expressing GP Ibα fragment, selectively killing cells with BCRs specific for GP Ibα General B cell depletion (Rituximab)

03

Biological functions

Immune response (specifically, generation of pathogenic autoantibodies in autoimmune disease)Cell activation (through antigen recognition)Maintenance/breakdown of self-tolerance
04

Disease associations

Autoimmune diseaseImmune thrombocytopenia (ITP)Other platelet destruction syndromes
05

Safety considerations

Risk of off-target B cell depletion (broad anti–B cell therapies)Immunosuppression leading to infection riskCytopenias and organ toxicity (in some cellular therapies)
06

Interacting drugs

GPIbα CAAR T cells

3 more in the full profile.

07

Biomarkers

Anti-GPIbα antibodies in plasma (indicate presence of autoreactive B cells)BCR specificity for GP Ibα (for research and selection of CAAR T therapy candidates)

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