| http://purl.uniprot.org/citations/29728406 | http://www.w3.org/1999/02/22-rdf-syntax-ns#type | http://purl.uniprot.org/core/Journal_Citation |
| http://purl.uniprot.org/citations/29728406 | http://www.w3.org/2000/01/rdf-schema#comment | "Allogeneic hematopoietic stem cell transplant (HSCT) typically results in donor T-cell engraftment and function in patients with severe combined immunodeficiency (SCID), but humoral immunity, particularly when using donors other than matched siblings, is variable. B-cell function after HSCT for SCID depends on the genetic cause, the use of pre-HSCT conditioning, and whether donor B-cell chimerism is achieved. Patients with defects in IL2RG or JAK3 undergoing HSCT without conditioning often have poor B-cell function post-HSCT, perhaps as a result of impairment of IL-21 signaling in host-derived B cells. To investigate the effect of pre-HSCT conditioning on B-cell function, and the relationship of in vitro B-cell function to clinical humoral immune status, we analyzed 48 patients with IL2RG/JAK3 SCID who were older than 2 years after HSCT with donors other than matched siblings. T follicular helper cells (TFH) developed in these patients with kinetics similar to healthy young children; thus, poor B-cell function could not be attributed to a failure of TFH development. In vitro differentiation of B cells into plasmablasts and immunoglobulin secretion in response to IL-21 strongly correlated with the use of conditioning, donor B-cell engraftment, freedom from immunoglobulin replacement, and response to tetanus vaccine. Patients receiving immunoglobulin replacement who had normal serum immunoglobulin M showed poor response to IL-21 in vitro, similar to those with low serum IgM. In vitro response of B cells to IL-21 may predict clinically relevant humoral immune function in patients with IL2RG/JAK3 SCID after HSCT."xsd:string |
| http://purl.uniprot.org/citations/29728406 | http://purl.org/dc/terms/identifier | "doi:10.1182/blood-2017-10-809822"xsd:string |
| http://purl.uniprot.org/citations/29728406 | http://purl.uniprot.org/core/author | "Notarangelo L.D."xsd:string |
| http://purl.uniprot.org/citations/29728406 | http://purl.uniprot.org/core/author | "Eibel H."xsd:string |
| http://purl.uniprot.org/citations/29728406 | http://purl.uniprot.org/core/author | "Hanson I.C."xsd:string |
| http://purl.uniprot.org/citations/29728406 | http://purl.uniprot.org/core/author | "Cowan M.J."xsd:string |
| http://purl.uniprot.org/citations/29728406 | http://purl.uniprot.org/core/author | "Puck J.M."xsd:string |
| http://purl.uniprot.org/citations/29728406 | http://purl.uniprot.org/core/author | "Whangbo J.S."xsd:string |
| http://purl.uniprot.org/citations/29728406 | http://purl.uniprot.org/core/author | "Kapoor N."xsd:string |
| http://purl.uniprot.org/citations/29728406 | http://purl.uniprot.org/core/author | "Buckley R.H."xsd:string |
| http://purl.uniprot.org/citations/29728406 | http://purl.uniprot.org/core/author | "Parrott R.E."xsd:string |
| http://purl.uniprot.org/citations/29728406 | http://purl.uniprot.org/core/author | "Decaluwe H."xsd:string |
| http://purl.uniprot.org/citations/29728406 | http://purl.uniprot.org/core/author | "Walter J.E."xsd:string |
| http://purl.uniprot.org/citations/29728406 | http://purl.uniprot.org/core/author | "Haddad E."xsd:string |
| http://purl.uniprot.org/citations/29728406 | http://purl.uniprot.org/core/author | "Pai S.Y."xsd:string |
| http://purl.uniprot.org/citations/29728406 | http://purl.uniprot.org/core/author | "O'Reilly R.J."xsd:string |
| http://purl.uniprot.org/citations/29728406 | http://purl.uniprot.org/core/author | "Abdel-Azim H."xsd:string |
| http://purl.uniprot.org/citations/29728406 | http://purl.uniprot.org/core/author | "Griffith L.M."xsd:string |
| http://purl.uniprot.org/citations/29728406 | http://purl.uniprot.org/core/author | "Kohn D.B."xsd:string |
| http://purl.uniprot.org/citations/29728406 | http://purl.uniprot.org/core/author | "Prockop S.E."xsd:string |
| http://purl.uniprot.org/citations/29728406 | http://purl.uniprot.org/core/author | "Hopkins G."xsd:string |
| http://purl.uniprot.org/citations/29728406 | http://purl.uniprot.org/core/author | "Davila Saldana B.J."xsd:string |
| http://purl.uniprot.org/citations/29728406 | http://purl.uniprot.org/core/author | "Dvorak C.C."xsd:string |
| http://purl.uniprot.org/citations/29728406 | http://purl.uniprot.org/core/author | "Gillio A."xsd:string |