The Experts below are selected from a list of 234 Experts worldwide ranked by ideXlab platform
John M. Routes - One of the best experts on this subject based on the ideXlab platform.
-
Newborn screening for T-Cell Deficiency.
Current Opinion in Allergy and Clinical Immunology, 2010Co-Authors: Nicole M. Chase, James W Verbsky, John M. RoutesAbstract:Purpose of reviewNewborn screening for T-Cell Deficiency is ongoing in Two sTaTes, and The published resulTs of 1 year of screening in Wisconsin are favorable. In This review, The hisTory, meThodology, resulTs, challenges, and fuTure direcTion of screening are discussed.RecenT findingsAs a concepT,
Dale I. Godfrey - One of the best experts on this subject based on the ideXlab platform.
-
AssociaTion beTween alphabeTaTCR+CD4-CD8- T-Cell Deficiency and IDDM in NOD/LT mice.
Diabetes, 1997Co-Authors: Alan G. Baxter, Simon J. Kinder, Kirsten J. L. Hammond, Roland Scollay, Dale I. GodfreyAbstract:NOD mice develop sponTaneous IDDM as a resulT of T-Cell-mediaTed auToimmune desTrucTion of pancreaTic beTa-Cells. IT is noT known why These T-Cells become auToreacTive, nor is iT clear wheTher The breakdown in self-Tolerance reflecTs a general problem in T-Cell developmenT or a selecTive defecT in an as yeT undefined regulaTory Cell populaTion. In This sTudy, we showed ThaT NOD mice, alThough relaTively normal wiTh regard To mosT ThymocyTe subseTs, exhibiT a marked Deficiency in alphabeTaTCR+CD4-CD8- (alphabeTa+DN) T-Cells in The Thymus and, To a lesser exTenT, in The periphery. These T-Cells have been Termed NKT Cells (NK1.1+-like T-Cells) because They share some Cell surface markers wiTh convenTional naTural killer (NK) Cells. To examine The role of These Cells in The paThogenesis of IDDM, semiallogeneic or syngeneic double-negaTive (DN) ThymocyTes, enriched for NKT Cells, were Transferred inTo inTacT 4-week-old NOD recipienTs; The onseT of diabeTes was Then moniTored over The ensuing 30 weeks. Mice receiving NKT-enriched ThymocyTes did noT develop diabeTes, whereas mice receiving unfracTionaTed ThymocyTes or phosphaTe-buffered saline developed diabeTes aT The normal raTe. NKT Cells represenT a disTincT T-Cell lineage ThaT has been shown To play a role in immunoregulaTion in vivo. The Deficiency of These Cells observed in NOD mice may Therefore conTribuTe To desTrucTion of pancreaTic isleT Cells by convenTional T-Cells.
-
associaTion beTween αβTcr cd4 cd8 T Cell Deficiency and iddm in nod lT mice
Diabetes, 1997Co-Authors: Alan G. Baxter, Simon J. Kinder, Kirsten J. L. Hammond, Roland Scollay, Dale I. GodfreyAbstract:NOD mice develop sponTaneous IDDM as a resulT of T-Cell-mediaTed auToimmune desTrucTion of pancreaTic β-Cells. IT is noT known why These T-Cells become auToreacTive, nor is iT clear wheTher The breakdown in self-Tolerance reflecTs a general problem in T-Cell developmenT or a selecTive defecT in an as yeT undefined regulaTory Cell populaTion. In This sTudy, we showed ThaT NOD mice, alThough relaTively normal wiTh regard To mosT ThymocyTe subseTs, exhibiT a marked Deficiency in αβTCR+CD4-CD8- (αβ+DN) T-Cells in The Thymus and, To a lesser exTenT, in The periphery. These T-Cells have been Termed NKT Cells (NK1.1+-like T-Cells) because They share some Cell surface markers wiTh convenTional naTural killer (NK) Cells. To examine The role of These Cells in The paThogenesis of IDDM, semiallogeneic or syngeneic double-negaTive (DN) ThymocyTes, enriched for NKT Cells, were Transferred inTo inTacT 4-week-old NOD recipienTs; The onseT of diabeTes was Then moniTored over The ensuing 30 weeks. Mice receiving NKT-enriched ThymocyTes did noT develop diabeTes, whereas mice receiving unfracTionaTed ThymocyTes or phosphaTe-buffered saline developed diabeTes aT The normal raTe. NKT Cells represenT a disTincT T-Cell lineage ThaT has been shown To play a role in immunoregulaTion in vivo. The Deficiency of These Cells observed in NOD mice may Therefore conTribuTe To desTrucTion of pancreaTic isleT Cells by convenTional T-Cells.
-
associaTion beTween alphabeTaTcr cd4 cd8 T Cell Deficiency and iddm in nod lT mice
Diabetes, 1997Co-Authors: Alan G. Baxter, Simon J. Kinder, Kirsten J. L. Hammond, Roland Scollay, Dale I. GodfreyAbstract:NOD mice develop sponTaneous IDDM as a resulT of T-Cell-mediaTed auToimmune desTrucTion of pancreaTic beTa-Cells. IT is noT known why These T-Cells become auToreacTive, nor is iT clear wheTher The breakdown in self-Tolerance reflecTs a general problem in T-Cell developmenT or a selecTive defecT in an as yeT undefined regulaTory Cell populaTion. In This sTudy, we showed ThaT NOD mice, alThough relaTively normal wiTh regard To mosT ThymocyTe subseTs, exhibiT a marked Deficiency in alphabeTaTCR+CD4-CD8- (alphabeTa+DN) T-Cells in The Thymus and, To a lesser exTenT, in The periphery. These T-Cells have been Termed NKT Cells (NK1.1+-like T-Cells) because They share some Cell surface markers wiTh convenTional naTural killer (NK) Cells. To examine The role of These Cells in The paThogenesis of IDDM, semiallogeneic or syngeneic double-negaTive (DN) ThymocyTes, enriched for NKT Cells, were Transferred inTo inTacT 4-week-old NOD recipienTs; The onseT of diabeTes was Then moniTored over The ensuing 30 weeks. Mice receiving NKT-enriched ThymocyTes did noT develop diabeTes, whereas mice receiving unfracTionaTed ThymocyTes or phosphaTe-buffered saline developed diabeTes aT The normal raTe. NKT Cells represenT a disTincT T-Cell lineage ThaT has been shown To play a role in immunoregulaTion in vivo. The Deficiency of These Cells observed in NOD mice may Therefore conTribuTe To desTrucTion of pancreaTic isleT Cells by convenTional T-Cells.
Chaim M. Roifman - One of the best experts on this subject based on the ideXlab platform.
-
HemaTopoieTic STem Cell TransplanTaTion for Profound T-Cell Deficiency (Combined ImmunoDeficiency)
Immunology and allergy clinics of North America, 2010Co-Authors: Chaim M. RoifmanAbstract:Typical cases of severe combined immunoDeficiency presenT aT infancy (mosT frequenTly aT 6 monThs of age) wiTh repeaTed opporTunisTic infecTions ; failure To Thrive; and scarciTy of lymphoid Tissues, including undeTecTable lymph nodes and a small dysplasTic Thymus. PaTienTs wiTh profound T-Cell dysfuncTion (PTD)/combined immunoDeficiency (CID) have moderaTe To large numbers of circulaTing auTologous lymphocyTes wiTh variable residual funcTion. These Cells may inTerfere wiTh proper engrafTmenT and may complicaTe The procedure of HSCT, hence The need for condiTioning. There is no immediaTe explanaTion for The exCellenT ouTcome of hemaTopoieTic sTem Cell TransplanTaTion (HSCT) for PTD/CID. HisTorically, proTocols for mismaTched relaTed donor HSCT did noT include condiTioning regimens, which could jeopardize engrafTmenT. Careful sTudies on The role of condiTioning, especially myeloablaTive condiTioning, should be conducTed in The fuTure. IT is possible ThaT in some genoTypes, relaTed idenTical donor can be accepTed by The recipienT wiTh liTTle or no condiTioning. UnTil such sTudies become insTrucTive, The proTocols in currenT use seem To provide exCellenT, alThough noT perfecT, ouTcome in paTienTs wiTh PTD/CID.
-
A muTaTion in zap-70 proTein Tyrosine kinase resulTs in a selecTive immunoDeficiency.
Journal of Clinical Immunology, 1995Co-Authors: Chaim M. RoifmanAbstract:We have previously described a new Type of selecTive T-Cell Deficiency characTerized by persisTenT infecTions reminiscenT of severe combined immunoDeficiency. We show here ThaT selecTive T-Cell Deficiency paTienTs carry a muTaTion ofzap-70 proTein Tyrosine kinase, resulTing in a loss of The acTiviTy of This kinase. The Thymus ofzap-70−/− paTienTs shows The presence of CD4CD8 double-posiTive Cells in The corTex, however, only CD4, and noT CD8, single-posiTive Cells are presenT in The medulla. Peripheral CD4+ T Cells from Thezap-70−/− paTienTs exhibiT markedly reduced Tyrosine phosphorylaTion, fail To produce inTerleukin-2, and do noT proliferaTe in response To T-Cell recepTor sTimulaTion by miTogens or anTigens. Thuszap-70 kinase appears To be indispensable for The developmenT of CD8 single-posiTive T Cells as well as for The signal TransducTion and funcTion of single-posiTive CD4 T Cells.
Nicole M. Chase - One of the best experts on this subject based on the ideXlab platform.
-
Newborn screening for T-Cell Deficiency.
Current Opinion in Allergy and Clinical Immunology, 2010Co-Authors: Nicole M. Chase, James W Verbsky, John M. RoutesAbstract:Purpose of reviewNewborn screening for T-Cell Deficiency is ongoing in Two sTaTes, and The published resulTs of 1 year of screening in Wisconsin are favorable. In This review, The hisTory, meThodology, resulTs, challenges, and fuTure direcTion of screening are discussed.RecenT findingsAs a concepT,
Alan G. Baxter - One of the best experts on this subject based on the ideXlab platform.
-
AssociaTion beTween alphabeTaTCR+CD4-CD8- T-Cell Deficiency and IDDM in NOD/LT mice.
Diabetes, 1997Co-Authors: Alan G. Baxter, Simon J. Kinder, Kirsten J. L. Hammond, Roland Scollay, Dale I. GodfreyAbstract:NOD mice develop sponTaneous IDDM as a resulT of T-Cell-mediaTed auToimmune desTrucTion of pancreaTic beTa-Cells. IT is noT known why These T-Cells become auToreacTive, nor is iT clear wheTher The breakdown in self-Tolerance reflecTs a general problem in T-Cell developmenT or a selecTive defecT in an as yeT undefined regulaTory Cell populaTion. In This sTudy, we showed ThaT NOD mice, alThough relaTively normal wiTh regard To mosT ThymocyTe subseTs, exhibiT a marked Deficiency in alphabeTaTCR+CD4-CD8- (alphabeTa+DN) T-Cells in The Thymus and, To a lesser exTenT, in The periphery. These T-Cells have been Termed NKT Cells (NK1.1+-like T-Cells) because They share some Cell surface markers wiTh convenTional naTural killer (NK) Cells. To examine The role of These Cells in The paThogenesis of IDDM, semiallogeneic or syngeneic double-negaTive (DN) ThymocyTes, enriched for NKT Cells, were Transferred inTo inTacT 4-week-old NOD recipienTs; The onseT of diabeTes was Then moniTored over The ensuing 30 weeks. Mice receiving NKT-enriched ThymocyTes did noT develop diabeTes, whereas mice receiving unfracTionaTed ThymocyTes or phosphaTe-buffered saline developed diabeTes aT The normal raTe. NKT Cells represenT a disTincT T-Cell lineage ThaT has been shown To play a role in immunoregulaTion in vivo. The Deficiency of These Cells observed in NOD mice may Therefore conTribuTe To desTrucTion of pancreaTic isleT Cells by convenTional T-Cells.
-
associaTion beTween αβTcr cd4 cd8 T Cell Deficiency and iddm in nod lT mice
Diabetes, 1997Co-Authors: Alan G. Baxter, Simon J. Kinder, Kirsten J. L. Hammond, Roland Scollay, Dale I. GodfreyAbstract:NOD mice develop sponTaneous IDDM as a resulT of T-Cell-mediaTed auToimmune desTrucTion of pancreaTic β-Cells. IT is noT known why These T-Cells become auToreacTive, nor is iT clear wheTher The breakdown in self-Tolerance reflecTs a general problem in T-Cell developmenT or a selecTive defecT in an as yeT undefined regulaTory Cell populaTion. In This sTudy, we showed ThaT NOD mice, alThough relaTively normal wiTh regard To mosT ThymocyTe subseTs, exhibiT a marked Deficiency in αβTCR+CD4-CD8- (αβ+DN) T-Cells in The Thymus and, To a lesser exTenT, in The periphery. These T-Cells have been Termed NKT Cells (NK1.1+-like T-Cells) because They share some Cell surface markers wiTh convenTional naTural killer (NK) Cells. To examine The role of These Cells in The paThogenesis of IDDM, semiallogeneic or syngeneic double-negaTive (DN) ThymocyTes, enriched for NKT Cells, were Transferred inTo inTacT 4-week-old NOD recipienTs; The onseT of diabeTes was Then moniTored over The ensuing 30 weeks. Mice receiving NKT-enriched ThymocyTes did noT develop diabeTes, whereas mice receiving unfracTionaTed ThymocyTes or phosphaTe-buffered saline developed diabeTes aT The normal raTe. NKT Cells represenT a disTincT T-Cell lineage ThaT has been shown To play a role in immunoregulaTion in vivo. The Deficiency of These Cells observed in NOD mice may Therefore conTribuTe To desTrucTion of pancreaTic isleT Cells by convenTional T-Cells.
-
associaTion beTween alphabeTaTcr cd4 cd8 T Cell Deficiency and iddm in nod lT mice
Diabetes, 1997Co-Authors: Alan G. Baxter, Simon J. Kinder, Kirsten J. L. Hammond, Roland Scollay, Dale I. GodfreyAbstract:NOD mice develop sponTaneous IDDM as a resulT of T-Cell-mediaTed auToimmune desTrucTion of pancreaTic beTa-Cells. IT is noT known why These T-Cells become auToreacTive, nor is iT clear wheTher The breakdown in self-Tolerance reflecTs a general problem in T-Cell developmenT or a selecTive defecT in an as yeT undefined regulaTory Cell populaTion. In This sTudy, we showed ThaT NOD mice, alThough relaTively normal wiTh regard To mosT ThymocyTe subseTs, exhibiT a marked Deficiency in alphabeTaTCR+CD4-CD8- (alphabeTa+DN) T-Cells in The Thymus and, To a lesser exTenT, in The periphery. These T-Cells have been Termed NKT Cells (NK1.1+-like T-Cells) because They share some Cell surface markers wiTh convenTional naTural killer (NK) Cells. To examine The role of These Cells in The paThogenesis of IDDM, semiallogeneic or syngeneic double-negaTive (DN) ThymocyTes, enriched for NKT Cells, were Transferred inTo inTacT 4-week-old NOD recipienTs; The onseT of diabeTes was Then moniTored over The ensuing 30 weeks. Mice receiving NKT-enriched ThymocyTes did noT develop diabeTes, whereas mice receiving unfracTionaTed ThymocyTes or phosphaTe-buffered saline developed diabeTes aT The normal raTe. NKT Cells represenT a disTincT T-Cell lineage ThaT has been shown To play a role in immunoregulaTion in vivo. The Deficiency of These Cells observed in NOD mice may Therefore conTribuTe To desTrucTion of pancreaTic isleT Cells by convenTional T-Cells.
