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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)
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: ubiquitous
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
Laboratory:
- BCL10 Ag in tissue
General
bcl protein (B-cell leukemia/lymphoma protein)
caspase recruitment domain protein (CARD protein)
Properties
SIZE: entity length = 233 aa
MW = 26 kD
COMPARTMENT: cytoplasm
cell nucleus
MOTIF: CARD domain {13-101}
Database Correlations
OMIM 603517
MORBIDMAP 603517
UniProt O95999
Pfam PF00619
Entrez Gene 8915
Kegg hsa:8915
References
- Entrez Gene :accession 8915
- UniProt :accession O95999