Abstract
Down syndrome is characterized by dysmorphic features, mental retardation and problems of immune deficiency. Chronic infection by Epstein–Barr virus is frequently present in subjects with Down syndrome. Ksp37 gene is commonly expressed by NK, CD8+ T, γδ T and CD4+ T cells; these data suggest that Ksp37 have cytotoxic properties. An increase of Ksp37 protein serum levels it has been showed during the acute phase of Epstein–Barr virus. In this study, we evaluated the expression of Ksp37 mRNA, in fibroblasts and leukocytes of DS subjects and in normal subjects with realtime reverse transcription-PCR. This analysis shows that in fibroblasts and leukocytes of Down syndrome subjects the KSP37 gene expression was increased compared with control subjects. The results of this study suggest that the expression of Ksp37 gene might be associated with increased susceptibility of individuals with Down syndrome to EBV infections and autoimmune problems.
References
Mrak RE, Griffin WS (2004) Trisomy 21 and the brain. J Neuropathol Exp Neurol 63:679–685
de Hingh YC, van der Vossen PW, Gemen EF et al (2005) Intrinsic abnormalities of lymphocyte counts in children with down syndrome. J Pediatr 147:744–747
Bull MJ (2011) Committee on Genetics. Health supervision for children with Down syndrome. Pediatrics 128:393–406
Kuitert PC, Abbink FC, Broers CJ et al (2014) Epstein–Barr virus infection with severe consequences. EBV, haemophagocytic lymphohistiocytosis and Hodgkin lymphoma with Down syndrome. Ned Tijdschr Geneeskd 158:A7608
Ogawa K, Tanaka K, Ishii A et al (2001) A novel serum protein that is selectively produced by cytotoxic lymphocytes. J Immun 166:6404–6412
Hayano C, Koi H, Ogawa K et al (2002) Accumulation of CD16+ cells with secretion of Ksp37 in decidua at the end of pregnancy. Am J Reprod Immun 48:57–62
Kuepper M, Koester K, Bratke K et al (2004) Increase in Ksp37-positive peripheral blood lymphocytes in mild extrinsic asthma. Clin Exp Immunol 137:359–365
Salemi M, Barone C, Romano C et al (2013) Pericentrin expression in Down’s syndrome. Neurol Sci 34:2023–2025
Romagnani S (2001) T-cell responses in allergy and asthma. Curr Opin Allergy Clin Immunol 1:73–78
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This study was supported by funds obtained from the Italian Ministry of Health (Ricerca Corrente).
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The authors declare that they have no conflict of interest.
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This study was approved by the Ethical Committee of the Research Institute “IRCCS Associazione Oasi Maria SS.”, Troina (EN), Italy. The Ethical Committee, chaired by Prof. Salvino Leone, approved the project on June 17, 2013 (Prot. N. CE2013/06/17).
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Salemi, M., Barone, C., Morale, M.C. et al. Killer-specific secretory (Ksp37) gene expression in subjects with Down’s syndrome. Neurol Sci 37, 793–795 (2016). https://doi.org/10.1007/s10072-016-2554-5
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DOI: https://doi.org/10.1007/s10072-016-2554-5