2022
Dysregulated stem cell niches and altered lymphocyte recirculation cause B and T cell lymphopenia in WHIM syndrome
Zehentmeier S, Lim VY, Ma Y, Fossati J, Ito T, Jiang Y, Tumanov AV, Lee HJ, Dillinger L, Kim J, Csomos K, Walter JE, Choi J, Pereira JP. Dysregulated stem cell niches and altered lymphocyte recirculation cause B and T cell lymphopenia in WHIM syndrome. Science Immunology 2022, 7: eabo3170. PMID: 36149943, PMCID: PMC9614684, DOI: 10.1126/sciimmunol.abo3170.Peer-Reviewed Original ResearchConceptsSecondary lymphoid organsWHIM syndromeMesenchymal stem cellsInterleukin-7B lymphopeniaBone marrowBM mesenchymal stem cellsT cell numbersIL-7 productionT-cell lymphopeniaLymphotoxin beta receptorEarly progenitor stageLymphoid organsCell lymphopeniaMouse modelBeta receptorsB cellsB cell developmentLymphopeniaStromal cellsLeukocyte retentionSyndromeGOF mutationsLymphopoietic activityCritical pathways
2020
Selective expression of claudin-5 in thymic endothelial cells regulates the blood–thymus barrier and T-cell export
Nagatake T, Zhao Y, Ito T, Itoh M, Kometani K, Furuse M, Saika A, Node E, Kunisawa J, Minato N, Hamazaki Y. Selective expression of claudin-5 in thymic endothelial cells regulates the blood–thymus barrier and T-cell export. International Immunology 2020, 33: 171-182. PMID: 33038259, PMCID: PMC7936066, DOI: 10.1093/intimm/dxaa069.Peer-Reviewed Original ResearchConceptsCortico-medullary junctionBlood-thymus barrierEndothelial cellsClaudin-5Biotin tracerCortical vasculatureBlood-borne moleculesCentral tolerance inductionT cell exportThymic endothelial cellsEC cell linesSphingosine-1-PhosphateT cell developmentDendritic cellsThymic cortexTolerance inductionTrans-endothelial resistanceThymocyte egressVascular permeabilityThymic microenvironmentMature thymocytesMedullaBlood circulationSelective expressionMedullary parenchyma