Recombination Activating Gene 1 (RAG1) ELISA Kits

Pig (Porcine) Recombination Activating Gene 1 (RAG1) interaction partners

1. Results confirm that Rag-1 is necessary for the generation of lymphocytes in pigs, and Rag-1-deficient piglets exhibit a T and B cell deficient severe combined immune deficiency(T-B-SCID (show PRKDC ELISA Kits)) phenotype similar to that of rodents and humans.

2. Piglets with biallelic mutations in either RAG1 or RAG2 (show RAG2 ELISA Kits) exhibit hypoplasia of immune organs, fail to perform V(D)J rearrangement, and lose mature B and T cells.

Human Recombination Activating Gene 1 (RAG1) interaction partners

1. 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.

2. Leaky SCID (show PRKDC ELISA Kits) can be caused by hypomorphic mutations in RAG1 and RAG2 (show RAG2 ELISA Kits) that result in only partial loss of enzymatic function of the proteins respectively encoded by these genes.

3. We report two siblings with SCID (show PRKDC ELISA Kits) and an atypical phenotype of osteopetrosis (OP (show CSF1 ELISA Kits)). A biallelic microdeletion encompassing the 5' region of TRAF6 (show TRAF6 ELISA Kits), RAG1 and RAG2 (show RAG2 ELISA Kits) genes was identified. TRAF6 (show TRAF6 ELISA Kits), a tumor necrosis factor (show TNF ELISA Kits) receptor-associated family member, plays an important role in T cell signaling and in RANKL (show TNFSF11 ELISA Kits)-dependent osteoclast differentiation and activation but its role in human OP has not been previously reported

4. The show that DNA damage caused by RAG1 activity in pre-B cells was able to downmodulate RAG1 expression and activity, confirming the existence of a negative feedback regulatory mechanism.

5. Our findings suggest that mutations in RAG1, specifically the p.R474C variant, can be associated with relatively mild clinical symptoms or delayed occurrence of T cell and B cell deficiencies but may predispose to progressive multifocal leukoencephalopathy.

6. This study compares T-cell development of patients with RAG1-dependent immune deficiencies, and elucidates important differences that help to explain the wide range of immunologic phenotypes that result from different mutations within the same gene of various patients.

7. Agammaglobulinemia associated to nasal polyposis due to a hypomorphic RAG1 mutation

8. mutation c.256_257delAA in RAG1 gene seems to occur quite frequently in the Polish patients with severe combined immunodeficiency (show PRKDC ELISA Kits) and may result in classical OS as well as in severe combined immunodeficiency (show PRKDC ELISA Kits) without clinical and laboratory features of OS when occurred in homozygous state

9. study reported 4 patients with RAG1 deficiency: classic severe combined immune deficiency (SCID (show PRKDC ELISA Kits)) was seen in 2 patients, Omenn syndrome was observed in 1 patient and atypical SCID (show PRKDC ELISA Kits) was seen in one patient

10. Study reports that RAG1 associates with chromatin at thousands of active promoters and enhancers in the genome of developing lymphocytes. The mouse and human genomes appear to have responded by reducing the abundance of "cryptic" recombination signals near RAG1 binding sites.

Mouse (Murine) Recombination Activating Gene 1 (RAG1) interaction partners

1. Describe spontaneous S. xylosus infection in a genetically modified murine model. S. xylosus infection in Rag1-/-Tpl2 (show MAP3K8 ELISA Kits)-/- mice correlated with disseminated bacteria and elevated numbers of circulating monocytes.

2. This study demonstrated that the lymphocyte-deficient Rag1 animals demonstrated a significantly lower rate of aneurysm formation and rupture.

3. This work provides important mechanistic insight into how spatiotemporal expression of the Rag genes is tightly controlled during B lymphocyte (show AKAP17A ELISA Kits) development to prevent mistimed dsDNA breaks and their deleterious consequences.

4. 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.

5. loss of the BH3-only (show BBC3 ELISA Kits) protein BIM (show BCL2L11 ELISA Kits) accelerated lymphoma development in p53 (show TP53 ELISA Kits)-deficient mice. This process was negated by concomitant loss of RAG1/2-mediated antigen receptor gene rearrangement.

6. Nonamer binding domain (NBD) of RAG1 plays a central role in the recognition of recombination signal sequence.

7. Overall, these findings suggest that the V(D)J recombination-activating gene 1, RAG1, may play a role in long-term memory consolidation.

8. propose that pre-B cells toggle between pre-BCR (show BCR ELISA Kits) signals and a RAG1 DNA double-strand break-dependent checkpoint to maintain genome stability while iteratively assembling Igl chain genes

9. Study reports that RAG1 associates with chromatin at thousands of active promoters and enhancers in the genome of developing lymphocytes. The mouse and human genomes appear to have responded by reducing the abundance of "cryptic" recombination signals near RAG1 binding sites.

10. The non-core region of RAG1 facilitates chromosomal V(D)J recombination in a ubiquitylation-dependent pathway.

Zebrafish Recombination Activating Gene 1 (RAG1) interaction partners

1. Differential gene expression following TLR stimulation in rag1-/- mutant zebrafish tissues and morphological descriptions of lymphocyte-like cell populations

2. first report of any rag1(-/-) mutant vertebrate mounting a protective secondary immune response to a bacterial pathogen

3. target-selected inactivation of the rag1 gene

4. Rag1 has a role in developing zebrafish cortex-medullary regionalization

5. by embryonic day 4, rag1 transcripts are seen in the pancreas

6. Recombination signal sequences are recognized by rag1 protein.

7. Rag1 expression is restricted to a subpopulation of zebrafish olfactory neurons projecting to the lateral olfactory bulb; Rag11 catalytic activity is not essential for axon targeting

8. This report describes the leukocyte populations in a unique model, recombination activation gene 1-/- mutant zebrafish (rag1 mutants).