TNFAIP3 (A20)

NF-kB negative regulator (tumour suppressor)

Expression
Deleted in ~50% (6q deletion)
Evidence level
strong

Role in pathogenesis

TNFAIP3 encodes A20, a key negative regulator of NF-kB signaling. Loss through chromosome 6q21-23 deletion occurs in ~50% of WM patients, removing the braking mechanism on NF-kB and synergising with MYD88 L265P to drive constitutive pathway activation.