Absence of the Thymus

George, AngeloM. Di; Lischner, HaroldW.; Dacou, Catherine; Arey, JamesB.

doi:10.1016/s0140-6736(67)91808-9

Cited by 43 publications

(21 citation statements)

References 12 publications

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“…Alternatively, previous authors have noted that the presence of immunoglobulins in some children with thymic defects is consistent with experimental evidence that the development of the immunoglobulin system is independent of the thymus (Di George et al, 1967). Neonatal thymectomy in several species impairs cell-mediated immune reactions, whereas hypo-y-globulinaemia has been produced only by ablation of other primary lymphoid organs Miller, 1966).…”

Section: Sibs Of 7 Children With Immunoglobulins Listed Insupporting

confidence: 61%

Thymic alymphoplasia, monoclonal gammopathy, and Pneumocystis carinii pneumonia in an infant.

Becroft¹,

Douglas²

1968

Archives of Disease in Childhood

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Section: Sibs Of 7 Children With Immunoglobulins Listed Insupporting

confidence: 61%

Thymic alymphoplasia, monoclonal gammopathy, and Pneumocystis carinii pneumonia in an infant.

Becroft¹,

Douglas²

1968

Archives of Disease in Childhood

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“…First publication on the syndrome comes from 1967 [8], describing children presenting with congenital heart malformation, hypoparathyreosis, thymus hypo/dysplasia or aplasia in rare cases of complete diGeorge syndrome, and other phenotypic features. The syndrome was later connected with deletion on chromosome 22 [9].…”

Section: Introductionmentioning

confidence: 99%

Helios Expression in T-regulatory Cells in Patients with di George Syndrome

Klocperk¹,

Grecová²,

Sismova³

et al. 2014

J Clin Immunol

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“…The thymic microenvironment is crucial for the maturation and expansion of T lymphocytes, the rearrangement of T-cell receptor genes, and the selection of T lymphocyte clones that are both responsive to foreign antigens and harmless to selftissues. Failure in generating a functional thymus, as in patients with the Di George syndrome and athymic nude mice, results in the loss of protection from microbial infection (Di George et al, 1967;Wortis et al, 1971). The thymus anlage is generated from the third pharyngeal pouch endoderm.…”

Section: Introductionmentioning

confidence: 99%

Mutations affecting thymus organogenesis in Medaka, Oryzias latipes

Iwanami

Takahama

Kunimatsu

et al. 2004

Mechanisms of Development

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The thymus is an organ for T lymphocyte maturation and is indispensable for the establishment of a highly developed immune system in vertebrates. In order to genetically dissect thymus organogenesis, we carried out a large-scale mutagenesis screening for Medaka mutations affecting recombination activating gene 1 (rag1) expression in the developing thymus. We identified 24 mutations, defining at least 13 genes, which led to a marked reduction of rag1 expression in the thymus. As thymus development depends on pharyngeal arches, we classified those mutations into three classes according to the defects in the pharyngeal arches. Class 1 mutants had no or slight morphological abnormalities in the pharyngeal arches, implying that the mutations may include defects in such thymus-specific events as lymphocyte development and thymic epithelial cell maturation. Class 2 mutants had abnormally shaped pharyngeal arches. Class 3 mutants showed severely attenuated pharyngeal arch development. In Class 2 and Class 3 mutants, the defects in thymus development may be due to abnormal pharyngeal arch development. Those mutations are expected to be useful for identifying the molecular mechanisms underlying thymus organogenesis.

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Absence of the Thymus

Cited by 43 publications

References 12 publications

Thymic alymphoplasia, monoclonal gammopathy, and Pneumocystis carinii pneumonia in an infant.

Thymic alymphoplasia, monoclonal gammopathy, and Pneumocystis carinii pneumonia in an infant.

Helios Expression in T-regulatory Cells in Patients with di George Syndrome

Mutations affecting thymus organogenesis in Medaka, Oryzias latipes

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