Defects in Thymocyte Differentiation and Thymocyte‐ Stromal Interactions in the Trisomy 16 Mouse

Ewart, Janet; Auerbach, Robert

doi:10.1155/1992/72627

Cited by 7 publications

(4 citation statements)

References 29 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…As some thymic and more generally immune defects have been also observed in trisomic 16 mice and partial trisomy 16 mice, models of trisomy 21 [23,60], which contain the SOD1 gene; our results can give some explanations regarding the immunological status of trisomy 21 persons suffering from high rate of Early thymic development in SOD1 transgenic mice.…”

Section: Discussionsupporting

confidence: 62%

Early thymic T cell development in young transgenic mice overexpressing human Cu/Zn superoxide dismutase, a model of Down syndrome

Laurent¹,

Paly

Marche

et al. 2006

Free Radical Biology and Medicine

View full text Add to dashboard Cite

. Early thymic T cell development in young transgenic mice overexpressing human Cu/Zn superoxide dismutase, a model of Down syndrome.: Early thymic development in SOD1 transgenic mice.. Free Radical Biology and Medicine, Elsevier, 2006Elsevier, , 40 (11), pp.1971Elsevier, -1980 ABSTRACTPrevious studies have shown that transgenic mice over-expressing Cu/Zn Superoxide dismutase, a model of Down's syndrome, exhibit premature thymic involution. We have performed an extensive multiparametric flow cytometry analysis of the developing thymus in these homozygous transgenic mice (hSOD1/hSOD1: Tg-SOD). Longitudinal follow-up analysis from day 3 to day 280 showed an early thymic development in Tg-SOD mice as compared with controls. This thymic early development was associated with an increased migration of mature T cells to peripheral lymphoid organs. BrdU labeling showed no difference between Tg-SOD and control mice, confirming that the greater number of peripheral T cells in Tg-SOD mice was not due to extensive proliferation of these cells but rather to a greater pool of emigrant T cells in Tg-SOD.

show abstract

Section: Discussionsupporting

confidence: 62%

Early thymic T cell development in young transgenic mice overexpressing human Cu/Zn superoxide dismutase, a model of Down syndrome

Laurent¹,

Paly

Marche

et al. 2006

Free Radical Biology and Medicine

View full text Add to dashboard Cite

show abstract

“…The fetal thymic development in the trisomy 16 (Ts16) mouse was examined, which is considered to be a model for human trisomy 21. The Ts16 thymus contains 10 to 20% of the number of lymphocytes found in a normal thymus at a comparable stage [ 31 ]. DS thymocytes have a markedly diminished proportion of cells expressing high levels of alpha, beta T cell receptor (alpha, beta TCR) and the associated CD3 molecule.…”

Section: Discussionmentioning

confidence: 99%

Inflammatory and Immunological parameters in adults with Down syndrome

et al. 2011

View full text Add to dashboard Cite

BackgroundThe increase in life expectancy within the general population has resulted in an increasing number of elderly adults, including patients with Down syndrome (DS), with a current life expectancy of about 50 years. We evaluate the parameters of humoral and cellular immune response, the quantitative expression of the regulator of calcineurin1 gene (RCAN1) and the production of cytokines. The study group consisted of adults DS (n = 24) and a control group with intellectual disability without Down syndrome (ID) (n = 21) and living in a similar environmental background. It was evaluated serology, immunophenotyping, the quantitative gene expression of RCAN1 and the production of cytokines.ResultsIn the DS group, the results showed an increase in NK cells, CD8, decreased CD19 (p < 0.05) and an increase spontaneous production of IFNgamma, TNFalpha and IL-10 (p < 0.05). There was not any difference in RCAN1 gene expression between the groups.ConclusionsThese data suggest a similar humoral response in the two groups. The immunophenotyping suggests sign of premature aging of the immune system and the cytokine production show a proinflammatory profile.

show abstract

“…The CTb model has recently been used to study amyloid aggregation 18 , endosomal abnormalities related to amyloid precursor protein 19 and apoptosis due to amyloid accumulation 20 . Similar to DS individuals, Ts16 mice exhibit congenital heart defects 21,22 , craniofacial and skeletal abnormalities, growth retardation 23 and immunological irregularities 24,25 . In addition, brain abnormalities similar to those in observed in DS occur.…”

Section: Introductionmentioning

confidence: 99%

Differential protein expression in normal and trisomic cell lines from murine cerebral cortex measured by itraq and Mass Spectrometry

et al. 2012

View full text Add to dashboard Cite

Down syndrome (DS), the most common cause of genetic mental dysfunction, is caused by aberrant protein expression due to a trisomy of chromosome 21 (Ts21). A mouse model of Ts21, based on synteny of human chromosome 21 and murine chromosome 16, is used for studying the mechanisms involved in DS. In this study we measured protein expression in immortalized cell lines derived from normal and trisomy 16 (Ts16) fetal mouse cerebral cortex (CNh and CTb respectively). iTRAQ labeling and mass spectrometry were used to examine each cell line for significant up- or down-regulation of proteins. CTb cells showed increased expression of 71 proteins and decreased expression of 56 proteins when compared to the normal CNh control cells. Several of these differentially expressed proteins have previously been reported to show aberrant expression in DS and other neurological disorders such as Alzheimerâ€™s disease, a condition closely associated with Down syndrome. Our results clearly demonstrate the aberrant expression of proteins with various cellular functions within the trisomy model and may lead to a deeper understanding of the proteins and mechanisms involved in the Down syndrome phenotype.

show abstract

Defects in Thymocyte Differentiation and Thymocyte‐ Stromal Interactions in the Trisomy 16 Mouse

Abstract: We have examined fetal thymic development in the trisomy 16

Cited by 7 publications

References 29 publications

Early thymic T cell development in young transgenic mice overexpressing human Cu/Zn superoxide dismutase, a model of Down syndrome

Early thymic T cell development in young transgenic mice overexpressing human Cu/Zn superoxide dismutase, a model of Down syndrome

Inflammatory and Immunological parameters in adults with Down syndrome

Differential protein expression in normal and trisomic cell lines from murine cerebral cortex measured by itraq and Mass Spectrometry

Contact Info

Product

Resources

About