| Description | 184 aa; a glycoprotein with a single transmembrane domain; N-terminally glycosylated at asparagine 42 regulates its function (Huang et al., 2013). BCMA is a signaling protein that activates the NF-kB. BCMA contains one cysteine rich domain and a DXL motif, comprised of (Phe/Tyr/Trp)-Asp-Xaa-Leu-(Val/Thr)-(Arg/Gly), to facilitate ligand binding with BAFF or APRIL (Hymowitz et al., 2005). |
| Expression | Preferentially expressed in lymph node tissue, B lymphoblast, NK cells, and dendritic cells. |
| Localisation | Plasma membrane. |
| Function | BCMA mediates the development and survival of B cell lymphocytes that maintain humoral immunity. |
| Homology | TNFRSF17 is conserved in P. Troglodytes, M. mulatta, C. lupus, B. taurus, M. musculus, and R. norvegicus. |
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| Entity | Multiple myeloma |
| Disease | Multiple myeloma is a fatal malignancy that involves the abnormal proliferation and accumulation of plasma cells characterized by their resistance to apoptosis. Sanchez et al. not only showed increased expression of BCMA in patients' plasma cells, but that patients with multiple myeloma have a high concentration of BCMA protein circulating in their blood. Furthermore, the group found that higher levels of circulating BCMA are associated with a worse prognosis. |
| Prognosis | Poor. |
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| Entity | Acute myelomonocytic leukemia (AMML) with eosinophilia |
| Disease | AMML is a malignancy involving myeloid precursors of the bone marrow. Although this condition is rare, it is a common type of pediatric acute myeloid leukemia. In about 50% of the cases of AMML, increased numbers of abnormal eosinophils are observed in the bone marrow (eosinophilia). AMML patients with eosinophilia who carry a pericentric inversion at 16(p13q22) tend to have a better prognosis. |
| Prognosis | 61% 5 year survival rate; AMML patients who carry the pericentric inversion have a higher rate of complete remission than those without the inversion (Chang et al., 2006). |
| Cytogenetics | Pericentric inversion at 16(p13q22) in 3-5% of AMML cases. |
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| Entity | T cell lymphoma |
| Note | (4;16)(q26;p13.1) chromosome translocation, which led to the discovery of BCMA, was noted by Laâbi et al. in a single case of T cell lymphoma. This translocation resulted in a IL-2/BCMA composite gene. |
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| Partial R-Banded karyotype. The karyotype shows a (4;16) (q26;p13.1) chromosomal translocation that led to the identification of BCMA by Laâbi et al. (Laâbi et al., 1992). |
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| Entity | Acute monocytic leukemia |
| Note | t(8;16)(p11;p13) translocation has been noted in cases of acute monocytic leukemia (Laâbi et al., 1992). |
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| Entity | Follicular lymphoma |
| Note | Translocation (16;18)(p13;q21.3) has been reported in follicular lymphoma (Mahmoodi et al., 2004). |
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| Acute myelomonocytic leukemia with abnormal eosinophils: a case report with multi-modality diagnostic work-up. |
| Chang ST, Hsieh YC, Lee LP, Tzeng CC, Chuang SS. |
| Chang Gung Med J. 2006 Sep-Oct;29(5):532-7. |
| PMID 17214400 |
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| B-cell maturation antigen is modified by a single N-glycan chain that modulates ligand binding and surface retention. |
| Huang HW, Chen CH, Lin CH, Wong CH, Lin KI. |
| Proc Natl Acad Sci U S A. 2013 Jul 2;110(27):10928-33. doi: 10.1073/pnas.1309417110. Epub 2013 Jun 17. |
| PMID 23776238 |
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| Structures of APRIL-receptor complexes: like BCMA, TACI employs only a single cysteine-rich domain for high affinity ligand binding. |
| Hymowitz SG, Patel DR, Wallweber HJ, Runyon S, Yan M, Yin J, Shriver SK, Gordon NC, Pan B, Skelton NJ, Kelley RF, Starovasnik MA. |
| J Biol Chem. 2005 Feb 25;280(8):7218-27. Epub 2004 Nov 12. |
| PMID 15542592 |
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| A new gene, BCM, on chromosome 16 is fused to the interleukin 2 gene by a t(4;16)(q26;p13) translocation in a malignant T cell lymphoma. |
| Laabi Y, Gras MP, Carbonnel F, Brouet JC, Berger R, Larsen CJ, Tsapis A. |
| EMBO J. 1992 Nov;11(11):3897-904. |
| PMID 1396583 |
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| Translocation (16;18)(p13;q21.3) in follicular lymphoma. |
| Mahmoodi M, Tanev SS, Punnett HH, Crilley P, Hou JS. |
| Cancer Genet Cytogenet. 2004 Oct 15;154(2):160-2. |
| PMID 15474153 |
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