A Prevalent CXCR3+ Phenotype of Circulating Follicular Helper T Cells Indicates Humoral Dysregulation in Children with Down Syndrome
Patients with Down syndrome (DS) are characterized by increased susceptibility to autoimmunity and respiratory tract infections that are suggestive of humoral immunity impairment. Here, we sought to determine the follicular helper (Tfh) and follicular regulatory (Tfr) T cell profile in the blood of...
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| Veröffentlicht in: | Journal of clinical immunology 2020-04, Vol.40 (3), p.447-455 |
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| creator | Ottaviano, Giorgio Gerosa, Jolanda Santini, Micaela De Leo, Pasqualina Vecchione, Andrea Jofra, Tatiana Trimarchi, Cristiana De Pellegrin, Maurizio Agosti, Massimo Aiuti, Alessandro Marinoni, Maddalena Cicalese, Maria Pia Fousteri, Georgia |
| description | Patients with Down syndrome (DS) are characterized by increased susceptibility to autoimmunity and respiratory tract infections that are suggestive of humoral immunity impairment. Here, we sought to determine the follicular helper (Tfh) and follicular regulatory (Tfr) T cell profile in the blood of children with DS. Blood was collected from 24 children with DS, nine of which had autoimmune diseases. Children with DS showed skewed Tfh differentiation towards the CXCR3
+
phenotype: Tfh1 and Tfh1/17 subsets were increased, while Tfh2 and Tfh17 subsets were reduced. While no differences in the percentage of Tfr cells were seen, the ratio of Tfh1 and CXCR3
+
PD-1
+
subsets to Tfr cells was significantly increased in the affected children. The excessive polarization towards a CXCR3
+
phenotype in children with DS suggests that re-calibration of Tfh subset skewing could potentially offer new therapeutic opportunities for these patients. |
| doi_str_mv | 10.1007/s10875-020-00755-0 |
| format | Article |
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+
phenotype: Tfh1 and Tfh1/17 subsets were increased, while Tfh2 and Tfh17 subsets were reduced. While no differences in the percentage of Tfr cells were seen, the ratio of Tfh1 and CXCR3
+
PD-1
+
subsets to Tfr cells was significantly increased in the affected children. The excessive polarization towards a CXCR3
+
phenotype in children with DS suggests that re-calibration of Tfh subset skewing could potentially offer new therapeutic opportunities for these patients.</description><identifier>ISSN: 0271-9142</identifier><identifier>EISSN: 1573-2592</identifier><identifier>DOI: 10.1007/s10875-020-00755-0</identifier><identifier>PMID: 31993866</identifier><language>eng</language><publisher>New York: Springer US</publisher><subject>Autoimmune diseases ; Biomedical and Life Sciences ; Biomedicine ; Children ; CXCR3 protein ; Down syndrome ; Down's syndrome ; Genotype & phenotype ; Humoral immunity ; Immunology ; Infectious Diseases ; Internal Medicine ; Lymphocytes T ; Medical Microbiology ; Original Article ; PD-1 protein ; Phenotypes ; Respiratory tract diseases</subject><ispartof>Journal of clinical immunology, 2020-04, Vol.40 (3), p.447-455</ispartof><rights>Springer Science+Business Media, LLC, part of Springer Nature 2020</rights><rights>Springer Science+Business Media, LLC, part of Springer Nature 2020.</rights><lds50>peer_reviewed</lds50><woscitedreferencessubscribed>false</woscitedreferencessubscribed><citedby>FETCH-LOGICAL-c375t-1ce8a07811b2531802c546d08f66f9f0b5028113858464a8e222c95f19f37ffa3</citedby><cites>FETCH-LOGICAL-c375t-1ce8a07811b2531802c546d08f66f9f0b5028113858464a8e222c95f19f37ffa3</cites><orcidid>0000-0001-7745-7517</orcidid></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><linktopdf>$$Uhttps://link.springer.com/content/pdf/10.1007/s10875-020-00755-0$$EPDF$$P50$$Gspringer$$H</linktopdf><linktohtml>$$Uhttps://link.springer.com/10.1007/s10875-020-00755-0$$EHTML$$P50$$Gspringer$$H</linktohtml><link.rule.ids>314,780,784,27924,27925,41488,42557,51319</link.rule.ids><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/31993866$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>Ottaviano, Giorgio</creatorcontrib><creatorcontrib>Gerosa, Jolanda</creatorcontrib><creatorcontrib>Santini, Micaela</creatorcontrib><creatorcontrib>De Leo, Pasqualina</creatorcontrib><creatorcontrib>Vecchione, Andrea</creatorcontrib><creatorcontrib>Jofra, Tatiana</creatorcontrib><creatorcontrib>Trimarchi, Cristiana</creatorcontrib><creatorcontrib>De Pellegrin, Maurizio</creatorcontrib><creatorcontrib>Agosti, Massimo</creatorcontrib><creatorcontrib>Aiuti, Alessandro</creatorcontrib><creatorcontrib>Marinoni, Maddalena</creatorcontrib><creatorcontrib>Cicalese, Maria Pia</creatorcontrib><creatorcontrib>Fousteri, Georgia</creatorcontrib><title>A Prevalent CXCR3+ Phenotype of Circulating Follicular Helper T Cells Indicates Humoral Dysregulation in Children with Down Syndrome</title><title>Journal of clinical immunology</title><addtitle>J Clin Immunol</addtitle><addtitle>J Clin Immunol</addtitle><description>Patients with Down syndrome (DS) are characterized by increased susceptibility to autoimmunity and respiratory tract infections that are suggestive of humoral immunity impairment. Here, we sought to determine the follicular helper (Tfh) and follicular regulatory (Tfr) T cell profile in the blood of children with DS. Blood was collected from 24 children with DS, nine of which had autoimmune diseases. Children with DS showed skewed Tfh differentiation towards the CXCR3
+
phenotype: Tfh1 and Tfh1/17 subsets were increased, while Tfh2 and Tfh17 subsets were reduced. While no differences in the percentage of Tfr cells were seen, the ratio of Tfh1 and CXCR3
+
PD-1
+
subsets to Tfr cells was significantly increased in the affected children. The excessive polarization towards a CXCR3
+
phenotype in children with DS suggests that re-calibration of Tfh subset skewing could potentially offer new therapeutic opportunities for these patients.</description><subject>Autoimmune diseases</subject><subject>Biomedical and Life Sciences</subject><subject>Biomedicine</subject><subject>Children</subject><subject>CXCR3 protein</subject><subject>Down syndrome</subject><subject>Down's syndrome</subject><subject>Genotype & phenotype</subject><subject>Humoral immunity</subject><subject>Immunology</subject><subject>Infectious Diseases</subject><subject>Internal Medicine</subject><subject>Lymphocytes T</subject><subject>Medical Microbiology</subject><subject>Original Article</subject><subject>PD-1 protein</subject><subject>Phenotypes</subject><subject>Respiratory tract 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Syndrome</title><author>Ottaviano, Giorgio ; Gerosa, Jolanda ; Santini, Micaela ; De Leo, Pasqualina ; Vecchione, Andrea ; Jofra, Tatiana ; Trimarchi, Cristiana ; De Pellegrin, Maurizio ; Agosti, Massimo ; Aiuti, Alessandro ; Marinoni, Maddalena ; Cicalese, Maria Pia ; Fousteri, Georgia</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-c375t-1ce8a07811b2531802c546d08f66f9f0b5028113858464a8e222c95f19f37ffa3</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>2020</creationdate><topic>Autoimmune diseases</topic><topic>Biomedical and Life Sciences</topic><topic>Biomedicine</topic><topic>Children</topic><topic>CXCR3 protein</topic><topic>Down syndrome</topic><topic>Down's syndrome</topic><topic>Genotype & phenotype</topic><topic>Humoral immunity</topic><topic>Immunology</topic><topic>Infectious Diseases</topic><topic>Internal Medicine</topic><topic>Lymphocytes T</topic><topic>Medical 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Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Ottaviano, Giorgio</au><au>Gerosa, Jolanda</au><au>Santini, Micaela</au><au>De Leo, Pasqualina</au><au>Vecchione, Andrea</au><au>Jofra, Tatiana</au><au>Trimarchi, Cristiana</au><au>De Pellegrin, Maurizio</au><au>Agosti, Massimo</au><au>Aiuti, Alessandro</au><au>Marinoni, Maddalena</au><au>Cicalese, Maria Pia</au><au>Fousteri, Georgia</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>A Prevalent CXCR3+ Phenotype of Circulating Follicular Helper T Cells Indicates Humoral Dysregulation in Children with Down Syndrome</atitle><jtitle>Journal of clinical immunology</jtitle><stitle>J Clin Immunol</stitle><addtitle>J Clin Immunol</addtitle><date>2020-04-01</date><risdate>2020</risdate><volume>40</volume><issue>3</issue><spage>447</spage><epage>455</epage><pages>447-455</pages><issn>0271-9142</issn><eissn>1573-2592</eissn><abstract>Patients with Down syndrome (DS) are characterized by increased susceptibility to autoimmunity and respiratory tract infections that are suggestive of humoral immunity impairment. Here, we sought to determine the follicular helper (Tfh) and follicular regulatory (Tfr) T cell profile in the blood of children with DS. Blood was collected from 24 children with DS, nine of which had autoimmune diseases. Children with DS showed skewed Tfh differentiation towards the CXCR3
+
phenotype: Tfh1 and Tfh1/17 subsets were increased, while Tfh2 and Tfh17 subsets were reduced. While no differences in the percentage of Tfr cells were seen, the ratio of Tfh1 and CXCR3
+
PD-1
+
subsets to Tfr cells was significantly increased in the affected children. The excessive polarization towards a CXCR3
+
phenotype in children with DS suggests that re-calibration of Tfh subset skewing could potentially offer new therapeutic opportunities for these patients.</abstract><cop>New York</cop><pub>Springer US</pub><pmid>31993866</pmid><doi>10.1007/s10875-020-00755-0</doi><tpages>9</tpages><orcidid>https://orcid.org/0000-0001-7745-7517</orcidid></addata></record> |
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| language | eng |
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| source | SpringerNature Journals |
| subjects | Autoimmune diseases Biomedical and Life Sciences Biomedicine Children CXCR3 protein Down syndrome Down's syndrome Genotype & phenotype Humoral immunity Immunology Infectious Diseases Internal Medicine Lymphocytes T Medical Microbiology Original Article PD-1 protein Phenotypes Respiratory tract diseases |
| title | A Prevalent CXCR3+ Phenotype of Circulating Follicular Helper T Cells Indicates Humoral Dysregulation in Children with Down Syndrome |
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