B-cell lymphoma/leukemia 10; B-cell CLL/lymphoma 10; Bcl-10; CED-3/ICH-1 prodomain homologous E10-like regulator; CIPER; CARD-containing molecule enhancing NF-kappa-B; cellular homolog of vCARMEN; cCARMEN; mammalian CARD-containing adapter molecule E10; mE10; cellular-E10; c-E10; CARD-like apoptotic protein; hCLAP (BCL10 CIPER CLAP)
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Function
- promotes apoptosis, pro-caspase-9 maturation & activation of NF-kappa-B via NIK & IKK
- may be an adapter protein between upstream TNFR1-TRADD-RIP complex & the downstream NIK-IKK-IKAP complex
- substrate for MALT1
- phosphorylation results in dissociation from TRAF2 & binding to BIRC2/c-IAP2
- self-associates by CARD-CARD interaction & forms a tight complex with MALT1
- interacts with other CARD-proteins such as CARD9, CARD10, CARD11 & CARD14
- binds caspase-9 with its C-terminal domain
- interacts with TRAF2 & BIRC2/c-IAP2
Structure
contains 1 CARD domain
Compartment
- cytoplasm, perinuclear region
- appears to have a perinuclear, compact & filamentous pattern of expression
- also found in the nucleus of several types of tumor cells
Expression
Pathology
- chromosomal translocation t(1;14)(p22;q32) involving BCL10 with IgH is recurrent in low-grade MALT lymphoma; although the BCL10/IgH translocation leaves the coding region of BCL10 intact, frequent BCL10 mutations may occur via the Ig somatic hypermutation mechanism resulting in nucleotide transitions
- mutations seen in some MALT lymphoma, follicular lymphoma, germ cell tumors & mesothelioma