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    BIOMARKER:

    BIRC3 deletion

    i
    Other names: BIRC3, API2, c-IAP2, cIAP2, hiap-1, MALT2, MIHC, RNF49, Baculoviral IAP repeat containing 3
    Entrez ID:
    330
    Related biomarkers:
    Expression
    Mutation
    Fusion
    almost3years
    Biological significance of monoallelic and biallelic BIRC3 loss in del(11q) chronic lymphocytic leukemia progression. (PubMed, Blood Cancer J)
    Moreover, we demonstrate ex vivo in primary cells that del(11q) cases including BIRC3 within their deleted region show evidence of non-canonical NF-κB activation which correlates with high BCL2 levels and enhanced sensitivity to venetoclax. Furthermore, our results show that BIRC3 mutations in del(11q) cells promote clonal advantage in vitro and accelerate leukemic progression in an in vivo xenograft model. Altogether, this work highlights the biological bases underlying disease progression of del(11q) CLL patients harboring BIRC3 deletion and mutation.
    Journal • IO biomarker
    |
    BCL2 (B-cell CLL/lymphoma 2) • BIRC3 (Baculoviral IAP repeat containing 3)
    |
    Chr del(11q) • BIRC3 mutation • BIRC3 deletion
    |
    Venclexta (venetoclax)
    over3years
    [VIRTUAL] Biological Impact of Monoallelic and Biallelic BIRC3 Loss in Del(11q) Chronic Lymphocytic Leukemia Progression (ASH 2020)
    Taken together, our results suggest that del(11q) CLL patients harboring BIRC3 mutations should be considered as a CLL subgroup at a high risk of progression that might benefit from venetoclax-based therapies. Funding: PI18/01500
    IO biomarker
    |
    BCL2L1 (BCL2-like 1) • BIRC3 (Baculoviral IAP repeat containing 3) • PTPRC (Protein Tyrosine Phosphatase Receptor Type C)
    |
    Chr del(11q) • BCL2 overexpression • BIRC3 mutation • BIRC3 deletion
    |
    Venclexta (venetoclax)