| Description | 233 amino acids with a molecular weight of 26252 Da. |
| Expression | BCL10 is expressed in all normal and malignant tissues. In the lymphoid tissue, it is highly expressed in the germinal center but low in the mantle zone, and intermediate in the marginal zone. BCL10 is likely to play a role in the normal development of the germinal center. |
| Localisation | BCL10 resides in the cytoplasm or perinuclear region of normal cells. |
| Function | BCL10 functions normally as a proapoptotic protein through caspase recruitment domain (CARD) at the animo terminal and activation of NF-kappaB pathway. This activity requires oligomerization via the CARD domain and interaction between BCL10 and other CARD domain containing proteins including CARD9, CARD10, CARD11 and CARD14. |
| Homology | Equine herpesvirus-2 E10 gene. |
| Note | |
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| Entity | 1p rearrangement/Non-Hodgkin lymphoma |
| Disease | Extranodal marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue (MALT lymphoma).
Phenotype stem cell origin: Marginal zone B-cells.
Epidemiology: Commonly seen in MALT lymphoma involving stomach (approximately 4%) and lung (approximately 9%). Uncommon in MALT lymphoma involving other sites.
Evolution: MALT lymphoma may evolve to diffuse large B-cell lymphoma. |
| Prognosis | Generally indolent. |
| Cytogenetics | In t(1;14)(p22;q32). The gene on 14q32 is IgH. The breakpoint on 1p22 involves a recurrent breakpoint upstream of the promoter of BCL10.
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| Hybrid/Mutated Gene | BCL10-IgH.
The translocation t(1;14)(p22;q32) is associated with frameshift mutation of BCL10 and truncation of BCL10 protein distal to CARD. The mutant type of BCL10 enhances cell survival and proliferation through activation of NF-kappaB pathway. MALT lymphomas without BCL10 rearrangement may also carry BCL10 mutation however. |
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| MALT lymphoma expressing BCL10 (courtesy of Dr. Du M-Q). |
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| Abnormal Protein | MALT lymphomas with t(1;14)(p22;q32) demonstrate strong nuclear BCL10 staining regardless of BCL10 mutation status. MALT lymphoma without t(1;14)(p22;q32) may also show strong nuclear staining.So a strong nuclear BCL10 staining is not always a presumptive evidence of t(1;14)(p22;q32). This pattern is different from the weak cytoplasmic expression observed in normal germinal center B-cells. |
| Oncogenesis | Loss of CARD domain through translocation and mutations lead to loss of proapoptotic activity. In addition, MALT1 and BCL10 may synergize in the activation of NF-kappaB leading to enhanced cell survival and downstream activation of anti-apoptotic/proliferative signals. |
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| Entity | Various Cancers |
| Disease | BCL10 mutations have also been described in follicular lymphoma, Sezary syndrome, malignant mesothelioma, germ cell tumor, and colon cancer. |
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| MALT lymphoma: recent advances in aetiology and molecular genetics. |
| Du M-Q. |
| J Clin Exp Hematop. 2007 Nov;47(2):31-42. |
| PMID 18040143 |
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| BCL10 gene mutation in lymphoma. |
| Du MQ, Peng H, Liu H, Hamoudi RA, Diss TC, Willis TG, Ye H, Dogan A, Wotherspoon AC, Dyer MJ, Isaacson PG. |
| Blood. 2000 Jun 15;95(12):3885-90. |
| PMID 10845924 |
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| Bcl10 is involved in t(1;14)(p22;q32) of MALT B cell lymphoma and mutated in multiple tumor types. |
| Willis TG, Jadayel DM, Du MQ, Peng H, Perry AR, Abdul-Rauf M, Price H, Karran L, Majekodunmi O, Wlodarska I, Pan L, Crook T, Hamoudi R, Isaacson PG, Dyer MJ. |
| Cell. 1999 Jan 8;96(1):35-45. |
| PMID 9989495 |
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| Inactivating mutations and overexpression of BCL10, a caspase recruitment domain-containing gene, in MALT lymphoma with t(1;14)(p22;q32). |
| Zhang Q, Siebert R, Yan M, Hinzmann B, Cui X, Xue L, Rakestraw KM, Naeve CW, Beckmann G, Weisenburger DD, Sanger WG, Nowotny H, Vesely M, Callet-Bauchu E, Salles G, Dixit VM, Rosenthal A, Schlegelberger B, Morris SW. |
| Nat Genet. 1999 May;22(1):63-8. |
| PMID 10319863 |
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