TY - JOUR. T1 - Retraction. T2 - The BCL11A transcription factor directly activates RAG gene expression and V(D)J recombination. [Molecular and Cellular Biology, 33, 9, (2013), (1768-1781)] doi: 10.1128/MCB.00987-12. AU - Lee, Baeck Seung. AU - Dekker, Joseph D.. AU - Lee, Bum Kyu. AU - Iyer, Vishwanath R.. AU - Sleckman, Barry P.. AU - Shaffer, Arthur L.. AU - Ippolito, Gregory C.. AU - Tucker, Philip W.. PY - 2017/11/1. Y1 - 2017/11/1. N2 - Analysis of our data indicated duplicate bands in Fig. 6E and 7A. It is unclear whether the source of these errors was accidental or purposeful. However, these errors did not change the conclusions of the paper. Nonetheless, we thank the journal for recognizing these errors, and we hereby retract the paper.. AB - Analysis of our data indicated duplicate bands in Fig. 6E and 7A. It is unclear whether the source of these errors was accidental or purposeful. However, these errors did not change the conclusions of the paper. Nonetheless, we thank the journal ...

Dobbs K, Tabellini G, Calzoni E, Patrizi O, Martinez P, Giliani SC, Moratto D, Al-Herz W, Cancrini C, Cowan M, Bleesing J, Booth C, Buchbinder D, Burns SO, Chatila TA, Chou J, Daza-Cajigal V, Ott de Bruin LM, de la Morena M, Di Matteo G, Finocchi A, Geha R, Goyal RK, Hayward A, Holland S, Huang CH, Kanariou MG, King A, Kaplan B, Kleva A, Kuijpers TW, Lee BW, Lougaris V, Massaad M, Meyts I, Morsheimer M, Neven B, Pai SY, Parvaneh N, Plebani A, Prockop S, Reisli I, Soh JY, Somech R, Torgerson TR, Kim YJ, Walter JE, Gennery AR, Keles S, Manis JP, Marcenaro E, Moretta A, Parolini S, Notarangelo LD. Natural Killer Cells from Patients with Recombinase-Activating Gene and Non-Homologous End Joining Gene Defects Comprise a Higher Frequency of CD56bright NKG2A+++ Cells, and Yet Display Increased Degranulation and Higher Perforin Content. Front Immunol. 2017; 8:798 ...

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Characterization of cells transferred to recipient Rag-1−/− mice.T cells enriched from pooled spleens of CD4−/− mice (Donor CD8+) were used for flow cyt

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Severe Combined Immunodeficiency (SCID), characterized by a profound decrease in both the number and function of T cells, is related to more than 20 different mutations. Recombination-activating gene (RAG) 1 and 2 seem to be two of the most common forms presenting with various manifestations, including typical SCID, Omenn syndrome (OS), atypical SCID (AS), or delayed onset combined immunodeficiency with granulomas. One interesting manifestation in RAG mutation is the change in the immunophenotype over time, even after hematopoietic stem cell transplantation (HSCT). As bone marrow transplantation (BMT) is the only curative treatment of SCID, it is necessary to differentiate between SCID and OS due to the different conditioning regimens (CR). We present a novel case of atypical SCID (SCID manifestations with more than 300 CD3+T cells) caused by RAG 2 gene mutation whose immunophenotype changed to atypical Omenn syndrome (all Omenn syndrome manifestations except rash, eosinophilia, and elevated IgE) over

V(D)J recombination is essential for developing lymphocytes and the specific functioning of the immune system. Germline gene coding segments become rearranged to create functional immunoglobulin and T-cell receptor genes by this recombination. The process depends on site-specific cleavage of chromosomal DNA by RAG-1 and RAG-2 recombinase. Two recombination-activating gene proteins (RAG-1/2) in conjunction make up a complex of enzymes that join gene segments of B-cell and T-cell receptor genes. RAG-1 contains most of the V(D)J recombinase active site and RAG-2 is essential in joining DNA segments during V(D)J recombination. RAG-1/2 have similarity in action to other DNA transposases and HIV-1 integrase. These similarities suggest that HIV-1 integrase inhibitors may have the potential to affect aspects of both B-cell and T-cell development.. Induction of primary immune responses to neoantigens involves the generation of specific T-cells and immunoglobulin M (IgM) antibody secreting B-cells. As ...

Mice. All mice were bred and housed under SPF conditions in the Centenary Institute Animal Facility, apart from BALB/cWehi and C.B-17/lcr-scid animals raised under GF or SPF conditions at the Walter & Eliza Hall Institute SPF Unit (Kew, Australia). Rag-1-/- mice (64) were originally a gift from Lynn Corcoran (Walter & Eliza Hall Institute, Parkville, Australia). Rag-2-/- mice (65) were purchased from Taconic Biosciences Inc. DEREG mice (37) were provided by Stephen Alexander (Childrens Hospital, Westmead, Australia). Tg mice expressing the 5C.C7 TCR specific for the COOH-terminal epitope of MCC (35, 36) were bred on a Rag-2-/- background. Cd28-/- mice were originally purchased from The Jackson Laboratory. Ctla4-/- mice (66) obtained from Jim Allison (MD Anderson Cancer Center, Houston, Texas, United States) were maintained as homozygotes on Rag-1-/- and Rag-2-/- backgrounds. F1 crosses were performed to generate immunosufficient (Rag-1+/-Rag-2+/-) Ctla4-/- BM donors. Experimental mice were ,8 ...

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The coordinated rearrangement of antigen receptor gene segments during V(D)J recombination is dependent on a complex series of DNA-processing reactions (20, 30, 45). Essential to the initiation of the process are recombination signal sequences (RSSs), which consist of two conserved DNA recognition motifs, the heptamer (consensus, CACAGTG) and the nonamer (consensus, ACAAAAACC) (32). These motifs are separated by predominantly nonconserved spacer regions of either 12 or 23 bp. Effective recombination is achieved by the 12/23 rule, which limits rearrangement to gene segments flanked by RSSs with different spacer lengths (15, 55, 60).. Recombination-activating genes 1 and 2 (RAG1 and RAG2) encode the lymphoid cell-specific recombinase components (36, 46) that are central to the rearrangement process. Normally, the V(D)J recombination reaction proceeds with nonamer recognition mediated by a DNA binding region of RAG1 (nonamer binding domain) that displays homology to the DNA recognition domains of ...

We developed a murine model of arterial gene transfer and used it to test the role of antigen-specific immunity in the loss of adenovirus-mediated transgene expression. Adenoviral vectors encoding either beta-galactosidase (beta-gal) or green fluorescent protein were infused to the lumen of normal common carotids of CD-1 and C57BL/6 mice and atherosclerotic carotids of Apoe(-/-) mice. At 3 days after gene transfer, significant reporter gene expression was detected in all strains. Transgene expression was transient, with expression undetectable at 14 days. Next, a beta-gal-expressing vector was infused into carotids of ROSA26 mice (transgenic for, and therefore tolerant of, beta-gal) and RAG-2(-/-) mice (deficient in recombinase-activating gene [RAG]-2 and therefore lacking in antigen-specific immunity). beta-Gal expression was again high at 3 days but declined substantially (|90%) by 14 days. In vivo labeling with bromodeoxyuridine revealed that carotid endothelial proliferation was in

Severe combined immune deficiency (SCID) is a group of genetic disorder associated with development of T- and/or B-lymphocytes. Recombination-activating genes (RAG1/2) play a critical role on VDJ recombinationDprocess that leads to the production of a broad T-cell receptor (TCR) and B-cell receptor (BCR) repertoire in the development of T and B cells. RAG1/2 genes mutations result in various forms of primary immunodeficiency, ranging from classic SCID to Omenn syndrome (OS) to atypical SCID with such as granuloma formation and autoimmunity. Herein, we reported 4 patients with RAG1 deficiency: classic SCID was seen in two patients who presented with recurrent pneumonia and chronic diarrhoea, and failure to thrive. OS was observed in one patient who presented with chronic diarrhoea, skin rash, recurrent lower respiratory infections, and atypical SCID was seen in one patient who presented with Pyoderma gangrenosum (PG) and had novel RAG1 mutation. ...

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Background Tuberculosis is one of the worlds leading killers, stealing 1. cell immune responses in infected NOS2-/-, RAG-/-, C3HeB/FeJ and C57/BL6 mice. Gr-1+ cells could be found on the edges of necrotic lung lesions in NOS2-/-, RAG-/-, and C3HeB/FeJ, but were absent in wild-type mice. Both populations of Gr1+CD11b+ cells expressed high levels of arginase-1, AMD 070 irreversible inhibition and IL-17, additional markers of myeloid derived suppressor cells. We then sorted the Gr1hi and Gr1int populations from infected NOS-/- mice and placed the sorted both Gr1int populations at different ratios with na?ve or infected splenocytes and evaluated their ability to induce activation and proliferation of CD4+T cells. Our results showed that both Gr1hi and Gr1int cells were able to induce activation and proliferation of CD4+ T cells. However this response was reduced as the ratio AMD 070 irreversible inhibition of CD4+ T to Gr1+ cells elevated. Our outcomes illustrate a however unrecognized interplay ...

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CD4(+)CD25(+) regulatory T (T(R)) cells can inhibit a variety of autoimmune and inflammatory diseases, but the precise mechanisms by which they suppress immune responses in vivo remain unresolved. Here, we have used Helicobacter hepaticus infection of T cell-reconstituted recombination-activating gene (RAG)(-/-) mice as a model to study the ability of CD4(+)CD25(+) T(R) cells to inhibit bacterially triggered intestinal inflammation. H. hepaticus infection elicited both T cell-mediated and T cell-independent intestinal inflammation, both of which were inhibited by adoptively transferred CD4(+)CD25(+) T(R) cells. T cell-independent pathology was accompanied by activation of the innate immune system that was also inhibited by CD4(+)CD25(+) T(R) cells. Suppression of innate immune pathology was dependent on T cell-derived interleukin 10 and also on the production of transforming growth factor beta. Thus, CD4(+)CD25(+) T(R) cells do not only suppress adaptive T cell responses, but are also able to control

Data_Sheet_1_K5 Capsule and Lipopolysaccharide Are Important in Resistance to T4 Phage Attack in Probiotic E. coli Strain Nissle 1917.pdf

Paton, T.A., A.J. Baker, J.G. Groth & G.F. Barrowclough. 2003. RAG-1 sequences resolve phylogenetic relationships within charadriiform birds. Molecular Phylogenetics Evolution 29(2): 268-278 ...

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This chapter on probiotic Escherichia coli focuses on the properties, underlying mechanisms, and clinical uses of Escherichia coli strain Nissle 1917 as this is the most widely used and studied strain. However, it also refers to important work that has been done using other E. coli strains. The enterobacterium E. coli can be extraintestinally pathogenic (e.g., uropathogenic), intestinally pathogenic (e.g., enteropathogenic or enterohemorrhagic), and nonpathogenic or commensal (e.g., probiotic). The probiotic E. coli strain Nissle 1917 was isolated in 1917 from a soldier who appeared to be protected from gastrointestinal infections causing severe diarrhea in many of his comrades. Since that time, EcN has been studied intensively, not only with a focus on its apparent clinical use but also with a view to understanding how it counteracts the pathogenic mechanisms underlying a number of diseases. Its uniqueness, not only among other E. coli strains but also among other probiotic microorganisms, is evident.

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ABAE cells (1.5 × 104) were plated in 24-well plates in 0.5 mL 10% FCS/DMEM medium. On the following day the cells were infected with 16K-Ad or Null-Ad at a multiplicity of infection (MOI) of 200 pfu/cell, and the conditioned media were collected 48 hours postinfection. Since the cDNA for 16K hPRL contains a signal peptide, 16K hPRL is secreted into the culture medium. Conditioned media (20 μL) was heated in sample buffer containing 1% 2-mercaptoethanol for 5 minutes at 95°C and separated by SDS/PAGE 15%/4% before being transferred to a nitrocellulose membrane (Hybond ECL; Amersham, Piscataway, NJ). The membrane was saturated overnight in TBS-8% dry milk, followed by 1 hour of incubation with a 1/200 dilution of polyclonal antiserum directed against 16K hPRL (SB30) and 1 hour of incubation with a 1/5000 dilution peroxidase-conjugated goat antirabbit serum (Gamma; BioWhittaker, Verviers, Belgium). 16K hPRL detection was then carried out by chemiluminescence using the ECL Plus kit (Amersham). ...

This article discusses partial T-cell disorders. For reviews of complete T-cell deficiencies, see the articles titled Severe Combined Immunodeficiency (SCID), Omenn Syndrome, and Cartilage-Hair Hypoplasia.

These NG-BAC transgenic mice express enhanced green fluorescent protein (EGFP) directed by the |i|Rag2|/i| promoter, and may be used in studies of B and T cell development as EGFP fluorescence reflects |i|Rag2|/i| protein expression.

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Cases reported • Severe Combined Immunodeficiency; Immunodeficiency, Severe Combined; Omenn Syndrome; Bare Lymphocyte Syndrome. On-line free medical diagnosis assistant. Ranked list of possible diseases from either several symptoms or a full patient history. A similarity measure between symptoms and diseases is provided.

possible extrathymic T cells (i.e. CD56þ T cells and CD57þ T Therefore, we used adrenalectomized mice in an attempt to cells) [9,10]. It was found that cell populations could be classified identify possible influences on the circadian rhythm [17]. These into two groups: one group with daytime rhythm includes granu- mice lost such circadian rhythms, implying some hormonal reg- locytes, monocytes, NK cells, extrathymic T cells, gd T cells, and ulation. However, because of impaired mobility, they also lost the CD8þ cells. The other, with night rhythm, includes conventional T variation of their physical activity round the clock. Changes in the and B cells, ab T cells, and CD4þ cells. It is well known that NK activity of the autonomic nervous system might affect leucocyte cells, extrathymic T cells and gd T cells are involved in natural subsets, some of them carrying adrenergic or cholinergic receptors immunity and are more primitive than conventional T (and ab T) [11,12]. There are many reports ...

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Looking for online definition of Omenn, Gilbert Stanley in the Medical Dictionary? Omenn, Gilbert Stanley explanation free. What is Omenn, Gilbert Stanley? Meaning of Omenn, Gilbert Stanley medical term. What does Omenn, Gilbert Stanley mean?

The RAG1/RAG2 (RAG) endonuclease recombines accessible antigen receptor (AgR) genes through DNA double strand break (DSB) intermediates to generate a diverse AgR repertoire. RAG-mediated DSBs signal changes in expression of genes encoding proteins involved in cellular survival, lymphocyte differentiation, and AgR selection. RAG proteins are each comprised of core endonuclease domains and dispensable non-core regions. Humans with mutations in non-core RAG1 regions exhibit fatal primary immunodeficiencies, and mice expressing truncated core, but not full-length, Rag1 protein (Rag1C/C mice) exhibit impaired early lymphocyte development associated with reduced levels of AgR gene rearrangements. In addition to serving with RAG2 as the V(D)J endonuclease, the RAG1 protein has been proposed to utilize non-core regions to regulate V(D)J recombination by enhancing AgR locus accessibility, promoting efficient RAG endonuclease activity, and/or generating RAG DSB-induced survival and differentiation signals.

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Screening detected 52 cases of typical SCID, leaky SCID, and Omenn syndrome, affecting 1 in 58,000 infants (95% CI, 1/46,000-1/80,000). Survival of SCID-affected infants through their diagnosis and immune reconstitution was 87% (45/52), 92% (45/49) for infants who received transplantation, enzyme replacement, and/or gene therapy. Additional interventions for SCID and non-SCID T-cell lymphopenia included immunoglobulin infusions, preventive antibiotics, and avoidance of live vaccines. Variations in definitions and follow-up practices influenced the rates of detection of non-SCID T-cell lymphopenia ...

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Simultaneous analysis of rag2 transcription and recombinase activity in B lineage progenitors. (A) Bone marrow obtained from the F1 progeny of RAG2 GFP × H2-SV

Bohlen, J., Slechtova, V., Tan H. H., & Britz, R. 2011. Phylogeny of the Southeast Asian freshwater fish genus Pangio (Cypriniformes; Cobitidae). Molecular Phylogenetics and Evolution, 61: 854-865.. http://www.sciencedirect.com/science/artic…055790311003472. Abstract. The genus Pangio is one of the most species-rich of the loach family Cobitidae and widespread across South and Southeast Asia. Its species diversity has never been studied under a clear phylogenetic approach, but four species-groups were proposed according to the most obvious morphological characters. We present here phylogenetic analyses of the genus Pangio based on sequence data of the mitochondrial cytochrome b gene, the nuclear recombination-activating gene 1 (RAG 1) and a combined dataset of 109 specimens from 18 morphologically identified species across the whole distribution area of the genus. Our data reveal the existence of three major lineages within Pangio. Two of our major lineages were congruent with formerly ...

Lymphoid V(D)J recombination: nucleotide insertion at signal joints as well as coding joints.: The coding regions of antigen receptor genes assembled by variabl

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