B-cell maturation antigen


B-cell maturation antigen, also known as tumor necrosis factor receptor superfamily member 17, is a protein that in humans is encoded by the TNFRSF17 gene.
TNFRSF17 is a cell surface receptor of the TNF receptor superfamily which recognizes B-cell activating factor.

Function

The protein encoded by this gene is a member of the TNF-receptor superfamily. This receptor is preferentially expressed in mature B lymphocytes, and may be important for B cell development and autoimmune response. This receptor has been shown to specifically bind to the tumor necrosis factor superfamily, member 13b, and to lead to NF-kappaB and MAPK8/JNK activation. This receptor also binds to various TRAF family members, and thus may transduce signals for cell survival and proliferation.

Interactions

TNFRSF17 has been shown to interact with the B-cell activating factor TNFSF13B. A conserved domain at the N-terminus, BCMA TALL-1 binding domain, is required for binding to the TNFSF13B.

Clinical significance - Related Diseases

TNFRSF17 is implicated in leukemia, lymphomas, and multiple myeloma .

As a drug target

In 2017, a phase 1 clinical trial employed a CAR-T therapy that targeted BCMA02 in multiple myeloma. Side-effects seen include cytokine release syndrome and mild neurotoxicity.
An antibody-drug conjugate GSK2857916 is in early clinical trials. Side effects are expected to include corneal toxicity.