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Gene Review

TSPYL2  -  TSPY-like 2

Homo sapiens

Synonyms: CDA1, CINAP, CTCL, CTCL-associated antigen se20-4, Cell division autoantigen 1, ...
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Disease relevance of TSPYL2


High impact information on TSPYL2

  • While in CBCL mostly indolent types displayed HLA-G positivity, in CTCL HLA-G expression was associated with high-grade histology and advanced stage of the disease [1].
  • Analysis of the T-cell receptor V beta repertoire in 14 CTCL patients found that only 4 had the expected monoclonal expansion of a specific V beta gene, whereas 10 had oligoclonal or polyclonal expansion of several V beta families [2].
  • Overexpression of hCINAP in HeLa cells results in a decrease in the average number of Cajal bodies per nucleus, consistent with it affecting either the stability of Cajal bodies and/or their rate of assembly [6].
  • The hCINAP protein sequence is highly conserved across its full-length from human to plants and yeast and is ubiquitously expressed in all human tissues and cell lines tested [6].
  • The yeast orthologue of CINAP is a single copy, essential gene [6].

Chemical compound and disease context of TSPYL2


Biological context of TSPYL2


Anatomical context of TSPYL2

  • Malignant lymphocytes in dermal infiltrates of CTCL tumors showed frequent and intense nuclear staining with anti-PY-STAT3 antibody, indicating a constitutive activation of STAT3 in vivo in tumor stages [11].
  • BACKGROUND AND DESIGN--Cutaneous T-cell lymphoma (CTCL) is a slowly advancing disease that initially presents in the skin and may later progress to involve the lymph nodes and viscera [12].
  • In the photopheresis apparatus, two synergistic phenomena are initiated: induction of apoptosis of the CTCL cells and mass conversion of blood monocytes to DCs [13].
  • Because curative therapies for CTCL are not yet available, short of TSEB in patients who have early-stage disease and allogeneic bone marrow transplantation in patients who have more advanced disease, the goal of current therapies is to prevent progression of MF and to preserve quality of life [14].
  • Identification of epitopes for CTCL-specific cytotoxic T lymphocytes [15].

Associations of TSPYL2 with chemical compounds

  • CDA1 comprises an N-terminal proline-rich domain, a central basic domain, and a C-terminal bipartite acidic domain [10].
  • There is fair evidence for the use of ECP in erythrodermic CTCL and steroid-refractory GVHD, but randomized controlled studies are needed [16].
  • Although milestone drugs such as bexarotene have been approved by the FDA- for treatment of CTCL, other agents such as IL-12 may also have a place in treatment of the disease [17].
  • The experimental treatment modalities, interferon-alpha and retinoids, especially arotinoid, are most effective in early stages of CTCL; in advanced stages, the effectiveness can be increased by combination regimens [18].
  • Hexadecylphosphocholine, a new, well-tolerated topical agent, induced a remission rate of 50%, with 25% complete remission in CTCL patients of stage Ia to IIb [18].

Other interactions of TSPYL2


Analytical, diagnostic and therapeutic context of TSPYL2

  • Both immunofluorescence and fluorescent protein tagging show that hCINAP is specifically nuclear and distributed in a widespread, diffuse nucleoplasmic pattern, excluding nucleoli, with some concentration also in Cajal bodies [6].
  • Immunohistochemistry revealed HLA-G protein expression in 23 (51%) of 45 cases (7 of 10 CBCL, 16 of 35 CTCL) [1].
  • Thus, we have defined a "molecular fingerprint" specific for a patient's malignant T-cells and can molecularly diagnose CTCL through PCR amplification [19].
  • CTA could provide defined targets for antigen-based vaccination in a high percentage of cases with CTCL [20].
  • Here, we studied signal transducers and activators of transcription (STAT) in patients with mycosis fungoides (MF)/cutaneous T-cell lymphoma (CTCL) [11].


  1. HLA-G protein up-regulation in primary cutaneous lymphomas is associated with interleukin-10 expression in large cell T-cell lymphomas and indolent B-cell lymphomas. Urosevic, M., Willers, J., Mueller, B., Kempf, W., Burg, G., Dummer, R. Blood (2002) [Pubmed]
  2. Association of erythrodermic cutaneous T-cell lymphoma, superantigen-positive Staphylococcus aureus, and oligoclonal T-cell receptor V beta gene expansion. Jackow, C.M., Cather, J.C., Hearne, V., Asano, A.T., Musser, J.M., Duvic, M. Blood (1997) [Pubmed]
  3. Combined modality treatment of cutaneous T cell lymphoma: results of a 6-year follow-up. Winkler, C.F., Sausville, E.A., Ihde, D.C., Fischmann, A.B., Schechter, G.P., Kumar, P.P., Nibhanupdi, J.R., Minna, J.D., Makuch, R.W., Eddy, J.L. J. Clin. Oncol. (1986) [Pubmed]
  4. Evaluation of a somatostatin analog in the treatment of lymphoproliferative disorders: results of a phase II North Central Cancer Treatment Group trial. Witzig, T.E., Letendre, L., Gerstner, J., Schroeder, G., Mailliard, J.A., Colon-Otero, G., Marschke, R.F., Windschitl, H.E. J. Clin. Oncol. (1995) [Pubmed]
  5. Chromosomal imbalances: a hallmark of tumour relapse in primary cutaneous CD30+ T-cell lymphoma. Prochazkova, M., Chevret, E., Beylot-Barry, M., Sobotka, J., Vergier, B., Delaunay, M., Turmo, M., Ferrer, J., Kuglik, P., Merlio, J.P. J. Pathol. (2003) [Pubmed]
  6. Characterization of hCINAP, a novel coilin-interacting protein encoded by a transcript from the transcription factor TAFIID32 locus. Santama, N., Ogg, S.C., Malekkou, A., Zographos, S.E., Weis, K., Lamond, A.I. J. Biol. Chem. (2005) [Pubmed]
  7. Thrombotic thrombocytopenic purpura-hemolytic uremic syndrome (TTP-HUS) following treatment with deoxycoformycin in a patient with cutaneous T-cell lymphoma (Sezary syndrome): A case report. Leach, J.W., Pham, T., Diamandidis, D., George, J.N. Am. J. Hematol. (1999) [Pubmed]
  8. Bexarotene and systemic disease progression in CTCL? Dummer, R., Hauschild, A., Skalsky, J., Burg, G., Weichenthal, M. J. Am. Acad. Dermatol. (2006) [Pubmed]
  9. Management of skin ulcers in a patient with mycosis fungoides. Panasiti, V., Devirgiliis, V., Borroni, R.G., Rossi, M., Curzio, M., Mancini, M., Bottoni, U., Calvieri, S. Dermatol. Online J. (2006) [Pubmed]
  10. SET-related cell division autoantigen-1 (CDA1) arrests cell growth. Chai, Z., Sarcevic, B., Mawson, A., Toh, B.H. J. Biol. Chem. (2001) [Pubmed]
  11. In vivo activation of STAT3 in cutaneous T-cell lymphoma. Evidence for an antiapoptotic function of STAT3. Sommer, V.H., Clemmensen, O.J., Nielsen, O., Wasik, M., Lovato, P., Brender, C., Eriksen, K.W., Woetmann, A., Kaestel, C.G., Nissen, M.H., Ropke, C., Skov, S., Ødum, N. Leukemia (2004) [Pubmed]
  12. Ultraviolet-B phototherapy for early-stage cutaneous T-cell lymphoma. Ramsay, D.L., Lish, K.M., Yalowitz, C.B., Soter, N.A. Archives of dermatology. (1992) [Pubmed]
  13. Cutaneous T cell lymphoma: the helping hand of dendritic cells. Edelson, R.L. Ann. N. Y. Acad. Sci. (2001) [Pubmed]
  14. Topical and systemic retinoid therapy for cutaneous T-cell lymphoma. Kempf, W., Kettelhack, N., Duvic, M., Burg, G. Hematol. Oncol. Clin. North Am. (2003) [Pubmed]
  15. Identification of epitopes for CTCL-specific cytotoxic T lymphocytes. Linnemann, T., Brock, C., Sparbier, K., Muche, M., Mielke, A., Lukowsky, A., Sterry, W., Kaltoft, K., Wiesmüller, K.H., Walden, P. Adv. Exp. Med. Biol. (1998) [Pubmed]
  16. Evidence-based practice of photopheresis 1987-2001: a report of a workshop of the British Photodermatology Group and the U.K. Skin Lymphoma Group. McKenna, K.E., Whittaker, S., Rhodes, L.E., Taylor, P., Lloyd, J., Ibbotson, S., Russell-Jones, R. Br. J. Dermatol. (2006) [Pubmed]
  17. Cutaneous T-cell lymphoma. New immunomodulators. Apisarnthanarax, N., Duvic, M. Dermatologic clinics. (2001) [Pubmed]
  18. Therapeutic approaches in cutaneous lymphoma. Jörg, B., Kerl, H., Thiers, B.H., Bröcker, E.B., Burg, G. Dermatologic clinics. (1994) [Pubmed]
  19. Molecular diagnosis of cutaneous T-cell lymphoma: polymerase chain reaction amplification of T-cell antigen receptor beta-chain gene rearrangements. Lessin, S.R., Rook, A.H., Rovera, G. J. Invest. Dermatol. (1991) [Pubmed]
  20. Expression of cancer/testis antigens in cutaneous T cell lymphomas. Häffner, A.C., Tassis, A., Zepter, K., Storz, M., Tureci, O., Burg, G., Nestle, F.O. Int. J. Cancer (2002) [Pubmed]
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