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000289207 1001_ $$00000-0001-6810-1607$$aGuérin, Antoine$$b0
000289207 245__ $$aHelper T cell immunity in humans with inherited CD4 deficiency.
000289207 260__ $$aNew York, NY$$bRockefeller Univ. Press$$c2024
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000289207 520__ $$aCD4+ T cells are vital for host defense and immune regulation. However, the fundamental role of CD4 itself remains enigmatic. We report seven patients aged 5-61 years from five families of four ancestries with autosomal recessive CD4 deficiency and a range of infections, including recalcitrant warts and Whipple's disease. All patients are homozygous for rare deleterious CD4 variants impacting expression of the canonical CD4 isoform. A shorter expressed isoform that interacts with LCK, but not HLA class II, is affected by only one variant. All patients lack CD4+ T cells and have increased numbers of TCRαβ+CD4-CD8- T cells, which phenotypically and transcriptionally resemble conventional Th cells. Finally, patient CD4-CD8- αβ T cells exhibit intact responses to HLA class II-restricted antigens and promote B cell differentiation in vitro. Thus, compensatory development of Th cells enables patients with inherited CD4 deficiency to acquire effective cellular and humoral immunity against an unexpectedly large range of pathogens. Nevertheless, CD4 is indispensable for protective immunity against at least human papillomaviruses and Trophyrema whipplei.
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000289207 7001_ $$00000-0002-3073-5345$$aMoncada-Vélez, Marcela$$b1
000289207 7001_ $$00000-0002-9952-7069$$aJackson, Katherine$$b2
000289207 7001_ $$00000-0003-2421-7389$$aOgishi, Masato$$b3
000289207 7001_ $$00000-0002-2822-161X$$aRosain, Jérémie$$b4
000289207 7001_ $$00000-0002-4981-7121$$aMancini, Mathieu$$b5
000289207 7001_ $$00000-0003-4429-0110$$aLanglais, David$$b6
000289207 7001_ $$00000-0002-0182-1834$$aNunez, Andrea$$b7
000289207 7001_ $$00000-0003-2494-5191$$aWebster, Samantha$$b8
000289207 7001_ $$00000-0002-1008-1890$$aGoyette, Jesse$$b9
000289207 7001_ $$00000-0002-7917-8965$$aKhan, Taushif$$b10
000289207 7001_ $$00000-0002-1927-7072$$aMarr, Nico$$b11
000289207 7001_ $$00000-0002-9156-0489$$aAvery, Danielle T$$b12
000289207 7001_ $$00009-0007-2744-9309$$aRao, Geetha$$b13
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000289207 7001_ $$00000-0002-7754-8109$$aNeves, Esmeralda$$b16
000289207 7001_ $$00000-0003-2814-9248$$aIracema Morais, Cátia$$b17
000289207 7001_ $$00000-0002-8857-111X$$aLondon, Jonathan$$b18
000289207 7001_ $$00009-0007-6427-6128$$aMestrallet, Stéphanie$$b19
000289207 7001_ $$00000-0002-1769-549X$$aQuartier Dit Maire, Pierre$$b20
000289207 7001_ $$00000-0001-8941-0935$$aNeven, Bénédicte$$b21
000289207 7001_ $$00000-0001-6553-2110$$aRapaport, Franck$$b22
000289207 7001_ $$00009-0000-2670-6026$$aSeeleuthner, Yoann$$b23
000289207 7001_ $$00000-0003-3416-1243$$aLev, Atar$$b24
000289207 7001_ $$00000-0003-0511-4122$$aSimon, Amos J$$b25
000289207 7001_ $$00000-0002-6983-4770$$aMontoya, Jorge$$b26
000289207 7001_ $$00000-0003-1168-9758$$aBarel, Ortal$$b27
000289207 7001_ $$00009-0007-9708-419X$$aGómez-Rodríguez, Julio$$b28
000289207 7001_ $$00000-0002-3809-9187$$aOrrego, Julio C$$b29
000289207 7001_ $$00000-0002-9228-0649$$aL'Honneur, Anne-Sophie$$b30
000289207 7001_ $$00009-0002-1445-7223$$aSoudée, Camille$$b31
000289207 7001_ $$00000-0002-5578-8160$$aRojas, Jessica$$b32
000289207 7001_ $$00000-0001-9157-8390$$aVelez, Alejandra C$$b33
000289207 7001_ $$00000-0002-0076-0224$$aSereti, Irini$$b34
000289207 7001_ $$00000-0001-6612-7336$$aTerrier, Benjamin$$b35
000289207 7001_ $$00000-0002-2626-3349$$aMarin, Nancy$$b36
000289207 7001_ $$00000-0002-5343-0050$$aGarcía, Luis F$$b37
000289207 7001_ $$00000-0001-7016-6493$$aAbel, Laurent$$b38
000289207 7001_ $$00000-0002-7115-116X$$aBoisson-Dupuis, Stéphanie$$b39
000289207 7001_ $$00000-0002-8007-2420$$aReis, Joel$$b40
000289207 7001_ $$aMarinho, Antonio$$b41
000289207 7001_ $$00000-0002-1475-356X$$aLisco, Andrea$$b42
000289207 7001_ $$00000-0002-9602-1599$$aFaria, Emilia$$b43
000289207 7001_ $$00000-0001-5296-6155$$aGoodnow, Christopher C$$b44
000289207 7001_ $$00000-0002-7315-5703$$aVasconcelos, Julia$$b45
000289207 7001_ $$00000-0002-4020-824X$$aBéziat, Vivien$$b46
000289207 7001_ $$00000-0001-5387-8413$$aMa, Cindy S$$b47
000289207 7001_ $$00000-0001-6648-9425$$aSomech, Raz$$b48
000289207 7001_ $$00000-0002-7782-4169$$aCasanova, Jean-Laurent$$b49
000289207 7001_ $$00000-0002-3439-2482$$aBustamante, Jacinta$$b50
000289207 7001_ $$00000-0001-5664-6415$$aFranco, Jose Luis$$b51
000289207 7001_ $$00000-0002-5360-5180$$aTangye, Stuart G$$b52
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