TNFRSF1B

Overview

TNFRSF1B (TNF Receptor Superfamily Member 1B, also known as TNFR2) encodes one of two receptors for tumor necrosis factor-alpha. Activating mutations in TNFRSF1B have been implicated in constitutive NFkB pathway activation in cutaneous T-cell lymphoma (CTCL), including Sézary syndrome and mycosis fungoides.

Alterations observed in the corpus

• p.Gly256Cys and p.Thr377Ile missense mutations in Sézary syndrome; consistent with prior reports of NFkB-activating TNFR2 alterations in mycosis fungoides and Sézary syndrome PMID:26551667

Cancer types (linked)

• SS (Sézary Syndrome): Recurrent p.Gly256Cys and p.Thr377Ile mutations; NFkB-activating alterations PMID:26551667
• MYCF (Mycosis Fungoides): Prior reports of similar NFkB-activating TNFR2 alterations (referenced in context of Sézary syndrome study) PMID:26551667

Co-occurrence and mutual exclusivity

• Convergence of CARD11 linker-domain mutations, TNFRSF1B mutations, and PRKG1 LoF on constitutive NFkB activation downstream of TCR signaling in CTCL PMID:26551667

Therapeutic relevance

• NFkB blockade (bortezomib, Mi-2 NFkB inhibitor) was highly active across all four CTCL cell lines regardless of specific genotype, consistent with convergent NFkB activation PMID:26551667

Open questions

Sources

• PMID:26551667

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