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Estimated disease incidence of RAG1/2 mutations: A case report and querying the Exome Aggregation Consortium.
Mutation of serine 365 to alanine permits bi-allelic and bi-locus RAG-mediated breaks in the same cell, leading to reciprocal translocations.
the ability of the RAG nuclease to minimize the risks of genome disruption by coupling the breakage and repair steps of the V(D)J reaction. This implies that the RAG genes, derived from an ancient transposon, have undergone strong selective pressure to prohibit transposition in favor of promoting controlled DNA end joining in cis by the ubiquitous DNA damage response and DNA repair machineries
We report two siblings with SCID and an atypical phenotype of osteopetrosis (OP). A biallelic microdeletion encompassing the 5' region of TRAF6, RAG1 and RAG2 genes was identified. TRAF6, a tumor necrosis factor receptor-associated family member, plays an important role in T cell signaling and in RANKL-dependent osteoclast differentiation and activation but its role in human OP has not been previously reported
The show that DNA damage caused by RAG2 activity in pre-B cells was able to downmodulate RAG2 expression and activity, confirming the existence of a negative feedback regulatory mechanism.
molecular and cellular mechanisms that account for the expanding range of clinical and immunological phenotypes of human RAG deficiency (review)
gene deficiency results in late onset autoimmune hemolytic anemia
This study reports on the prevalence of RAG1 and RAG2 mutations in ten severe combined immunodeficiency disorder patients in Egypt.
analysis of regions of RAG1 necessary for interaction with RAG2 and measurement of the RAG1-RAG2 binding affinity
DNA damage triggers relocalization of RAG2 from the nucleus to centrosomes, suggesting a novel mechanism for modulating cellular responses to double strand breaks in developing lymphocytes.
analysis of individual molecular events of RAG-mediated V(D)J DNA cleavage
found that Ikaros, a lymphocyte-specific transcription factor, acts as a repressor of NWC promoter--thus identifying a new Ikaros target--but is insufficient for inducing methylation which depends on the antisense transcription driven by RAG-2 promoter
Investigate the factors that regulate RAG1 and RAG2 cleavage on non-B DNA structures. We find that RAG binding and cleavage on heteroduplex DNA is dependent on the length of the double-stranded flanking region.
Observations indicate that the RAG proteins exert fine control over every step of V(D)J cleavage.
The results indicate that the contribution of immune dysregulatory disease due to RAG2 mutations present in the general population may be much higher than previously estimated.
Bidirectional activity of the NWC promoter is responsible for RAG-2 transcription in non-lymphoid cells
A novel homozygous mutation with different clinical phenotypes: Omenn syndrome and hyper-IgM syndrome
analysis of multiorgan metastasis of human HER-2+ breast cancer in Rag2-/-;Il2rg-/- mice and treatment with PI3K inhibitor
Homozygous mutation of p.R394Q/p.R394Q and p.R776Q, 3047-3049 del GCC mutations are novel and they are causing serious T-B-NK + SCID.
USF-1 but not USF-2 is strongly enriched at Dbeta2 in chromatin from either Rag2-/- deficient thymocytes or Rag2-/- deficient thymocytes that express a rearranged Tcrb transgene.
This work provides important mechanistic insight into how spatiotemporal expression of the Rag genes is tightly controlled during B lymphocyte development to prevent mistimed dsDNA breaks and their deleterious consequences.
a novel RAG1/2-mediated insertion pathway distinct from DNA transposition and trans-V(D)J recombination that destabilizes the genome and shares features with reported oncogenic DNA insertions.
loss of the BH3-only protein BIM accelerated lymphoma development in p53-deficient mice. This process was negated by concomitant loss of RAG1/2-mediated antigen receptor gene rearrangement.
the zinc finger motif within the noncore region of RAG2 is indispensable for maintaining the stability of the RAG2 protein.
evolution of RAG1/RAG2 began with a Transib transposon whose intrinsic recombination activity was enhanced by capture of an ancestral RAG2, allowing for the development of adaptive immunity.
Rag2(R229Q) knock-in mice developed an inflammatory bowel disease affecting both the small bowel and colon.
Rag2 truncation substantially increased the frequency of off-target V(D)J recombination. The data suggest that interactions between Rag2 and a specific chromatin modification, H3K4me3, support V(D)J recombination fidelity.
These results reveal an unanticipated functional interplay between the RAG complex and XLF in repairing RAG-induced DNA breaks and maintaining genome integrity during antigen receptor gene assembly.
Based on our results, we propose that interaction of RAG2 with RAG1 induces cooperative interactions of multiple binding sites, induced through conformational changes at the RAG1 interdomain boundary
12recombination signal sequences-23recombination signal sequences cooperation (the "12/23 rule") is important not only for regulating RAG-mediated DNA cleavage but also for the efficiency of RAG1 and RAG2 recruitment to chromatin.
This study demonstrated that The behavioral impairments in Rag2(-/-) mice were paralleled by an elevation in plasma corticosterone after behavioral tests.
We have confirmed the activation of human peripheral blood lymphocytes after being xenografted in immunodeficient Rag2-/-IL2Rgnull mice.
V(D)J recombination is stimulated by binding of H3K4me3 to RAG-2
The complex RAG1/2 binds one copy each of 12 recombination signal sequence and 23 recombination signal sequence DNA.
SATB1 binds to the ASE and Rag promoters, facilitating inclusion of Rag2 in the chromatin hub and the loading of RNA polymerase II to both the Rag1 and Rag2 promoters.
Study provides evidence that RAG expression (RAG1, RAG2)in uncommitted hematopoietic progenitors and NK cell precursors marks functionally distinct subsets of NK cells in the periphery, demonstrating both a novel role for RAG in the functional specialization of the NK cell lineage and a physiological role for RAG proteins outside of V(D)J recombination.
crystal structure of the mouse RAG1-RAG2 complex at 3.2 A resolution
Rag2-/-Il1rn-/- mice develop spontaneous severe colitis with high mortality.
Integrated genetic approaches identify the molecular mechanisms of Sox4 in early B-cell development: intricate roles for RAG1/2 and CK1epsilon.
Nod/Ripk2 signaling in dendritic cells activates IL-17A-secreting innate lymphoid cells and drives colitis in T-bet-/-.Rag2-/- (TRUC) mice.
Produced RAG2-knockouts via somatic cell nuclear transfer and analyzed their phenotype. The V(D)J recombination processes were confirmed as being inactivated. They consistently lacked mature T and B cells but had substantial numbers of cells considered to be T- or B-cell progenitors as well as NK cells. They also lacked thymic medulla and lymphoid aggregations in the spleen, mesenteric lymph nodes, and Peyer's patches.
Homozygous RAG1/2 knockout pigs lack mature B and T lymphocytes; recombination does not occur in the Ig heavy chain (IgH) locus of RAG1- and RAG2-deficient pigs.
created rag2(E450fs) mutant zebrafish that have reduced numbers of functional T and B cells but are viable and fecund
This gene encodes a protein that is involved in the initiation of V(D)J recombination during B and T cell development. This protein forms a complex with the product of the adjacent recombination activating gene 1, and this complex can form double-strand breaks by cleaving DNA at conserved recombination signal sequences. The recombination activating gene 1 component is thought to contain most of the catalytic activity, while the N-terminal of the recombination activating gene 2 component is thought to form a six-bladed propeller in the active core that serves as a binding scaffold for the tight association of the complex with DNA. A C-terminal plant homeodomain finger-like motif in this protein is necessary for interactions with chromatin components, specifically with histone H3 that is trimethylated at lysine 4. Mutations in this gene cause Omenn syndrome, a form of severe combined immunodeficiency associated with autoimmune-like symptoms.
recombination activating gene 2
, recombination activating protein 2
, V(D)J recombination-activating protein 2-like
, V(D)J recombination-activating protein 2
, recombinase activating gene 2
, v(D)J recombination-activating protein 2