RELA
Overview
RELA (v-rel avian reticuloendotheliosis viral oncogene homolog A), also known as NF-kB p65, is a transcription factor and central mediator of the NF-kB signaling pathway. In cancer, RELA is most notably involved as a fusion partner in supratentorial ependymoma, where C11orf95-RELA fusions define a distinct molecular subtype with poor prognosis.
Alterations observed in the corpus
- C11orf95-RELA fusion with chromothripsis-like alternating gains/losses on chromosomes 11 and 22 in supratentorial ependymoma (EPM) — diagnostic of RELA-type supratentorial ependymoma and prognostic for poor outcome; identified in a pediatric precision-oncology program (n=101 high-risk patients) PMID:28007021
Cancer types (linked)
- Ependymoma (EPM): C11orf95-RELA fusion is diagnostic of RELA-type supratentorial ependymoma; associated with poor prognosis PMID:28007021
Co-occurrence and mutual exclusivity
- C11orf95-RELA fusion co-occurs with chromothripsis-like structural rearrangements on chr11 and chr22 in ependymoma PMID:28007021
Therapeutic relevance
- No targeted therapy against RELA-fusion ependymoma is established in this corpus; identification of the fusion supported molecular subtyping relevant to prognosis counseling and potential NF-kB-targeted trial eligibility PMID:28007021
Open questions
- Whether NF-kB pathway inhibitors can provide clinical benefit in RELA-fusion supratentorial ependymoma remains under investigation.
Sources
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