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dc.contributor.author | Menezes, Soraya Maria | |
dc.contributor.author | Leal, Fabio E. | |
dc.contributor.author | Dierckx, Tim | |
dc.contributor.author | Khouri, Ricardo | |
dc.contributor.author | Decanine, Daniele | |
dc.contributor.author | Silva-Santos, Gilvaneia | |
dc.contributor.author | Schnitman, Saul V. | |
dc.contributor.author | Kruschewsky, Ramon | |
dc.contributor.author | Lopez, Giovanni | |
dc.contributor.author | Alvarez, Carolina | |
dc.contributor.author | Talledo Albujar, Michael John | |
dc.contributor.author | Gotuzzo Herencia, José Eduardo | |
dc.contributor.author | Nixon, Douglas F. | |
dc.contributor.author | Vercauteren, Jurgen | |
dc.contributor.author | Brassat, David | |
dc.contributor.author | Liblau, Roland | |
dc.contributor.author | Vandamme, Anne Mieke | |
dc.contributor.author | Galvao-Castro, Bernardo | |
dc.contributor.author | Van Weyenbergh, Johan | |
dc.date.accessioned | 2019-01-25T15:02:14Z | |
dc.date.available | 2019-01-25T15:02:14Z | |
dc.date.issued | 2017 | |
dc.identifier.uri | https://hdl.handle.net/20.500.12866/4585 | |
dc.description.abstract | Human T-cell lymphotropic virus (HTLV)-1 was the first human retrovirus to be associated to cancer, namely adult T-cell leukemia (ATL), but its pathogenesis remains enigmatic, since only a minority of infected individuals develops either ATL or the neuroinflammatory disorder HTLV-1-associated myelopathy/tropical spastic paraparesis (HAM/TSP). A functional FAS -670 polymorphism in an interferon (IFN)-regulated STAT1-binding site has been associated to both ATL and HAM/TSP susceptibility. Fashi T stem cell memory (Tscm) cells have been identified as the hierarchical apex of ATL, but have not been investigated in HAM/TSP. In addition, both FAS and STAT1 have been identified in an IFN-inducible HAM/TSP gene signature, but its pathobiological significance remains unclear. We comprehensively explored Fas expression (protein/mRNA) and function in lymphocyte activation, apoptosis, proliferation, and transcriptome, in PBMC from a total of 47 HAM/TSP patients, 40 asymptomatic HTLV-1-infected individuals (AC), and 58 HTLV-1 -uninfected healthy controls. Fas surface expression followed a two-step increase from HC to AC and from AC to HAM/TSP. In HAM/TSP, Fas levels correlated positively to lymphocyte activation markers, but negatively to age of onset, linking Fashi cells to earlier, more aggressive disease. Surprisingly, increased lymphocyte Fas expression in HAM/TSP was linked to decreased apoptosis and increased lymphoproliferation upon in vitro culture, but not to proviral load. This Fashi phenotype is HAM/TSP-specific, since both ex vivo and in vitro Fas expression was increased as compared to multiple sclerosis (MS), another neuroinflammatory disorder. To elucidate the molecular mechanism underlying non-apoptotic Fas signaling in HAM/TSP, we combined transcriptome analysis with functional assays, i.e., blocking vs. triggering Fas receptor in vitro with antagonist and agonist-, anti-Fas mAb, respectively. Treatment with agonist anti-Fas mAb restored apoptosis, indicating biased, but not defective Fas signaling in HAM/TSP. In silico analysis revealed biased Fas signaling toward proliferation and inflammation, driven by RelA/NF-κB. Correlation of Fas transcript levels with proliferation (but not apoptosis) was confirmed in HAM/TSP ex vivo transcriptomes. In conclusion, we demonstrated a two-step increase in Fas expression, revealing a unique Fashi lymphocyte phenotype in HAM/TSP, distinguishable from MS. Non-apoptotic Fas signaling might fuel HAM/TSP pathogenesis, through increased lymphoproliferation, inflammation, and early age of onset. | en_US |
dc.language.iso | eng | |
dc.publisher | Frontiers Media | |
dc.relation.ispartofseries | Frontiers in Immunology | |
dc.rights | info:eu-repo/semantics/restrictedAccess | |
dc.rights.uri | https://creativecommons.org/licenses/by-nc-nd/4.0/deed.es | |
dc.subject | apoptosis | en_US |
dc.subject | Fas/CD95 | en_US |
dc.subject | HTLV-1-associated myelopathy/tropical spastic paraparesis | en_US |
dc.subject | interferon | en_US |
dc.subject | lymphoproliferative disease | en_US |
dc.subject | multiple sclerosis | en_US |
dc.subject | NF-kappaB | en_US |
dc.subject | proliferation | en_US |
dc.title | A Fas(hi) Lymphoproliferative Phenotype Reveals Non-Apoptotic Fas Signaling in HTLV-1-Associated Neuroinflammation | en_US |
dc.type | info:eu-repo/semantics/article | |
dc.identifier.doi | https://doi.org/10.3389/fimmu.2017.00097 | |
dc.subject.ocde | https://purl.org/pe-repo/ocde/ford#3.01.03 | |
dc.relation.issn | 1664-3224 |
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