Abstract:
:DNA breaks can be induced by exogenous stimuli or by endogenous stress, but are also generated during recombination of V, D, and J genes (V(D)J recombination), immunoglobulin class switch recombination (CSR). Among various DNA breaks generated, DNA double strand break (DSB) is the most deleterious one. DNA damage response (DDR) is initiated when DSBs are detected, leading to DNA break repair by non-homologous end joining (NHEJ). The process is critically important for the generation of diversity for foreign antigens; and failure to exert DNA repair leads to immunodeficiency such as severe combined immunodeficiency and hyper-IgM syndrome. In V(D)J recombination, DSBs are induced by RAG1/2; and generated post-cleavage hairpins are resolved by Artemis/DNA-PKcs/KU70/KU80. DDR is initiated by ataxia-telangiectasia mutated as a master regulator together with MRE11/RAD50/NBS1 complex. Finally, DSBs are repaired by NHEJ. The defect of one of the molecules shows various degree of immunodeficiency and radiosensitivity. Upon CSR inducing signal, DSBs induced by activation-induced cytidine deaminase and endonucleases elicit DDR. Broken ends are repaired either by NHEJ or by mismatch repair system. Patients with radiosensitive SCID require hematopoietic cell transplantation as a curative therapy; but the procedures for eradication of recipient hematopoietic cells are often associated with severe toxicity.
journal_name
Int J Hematoljournal_title
International journal of hematologyauthors
Morio Tdoi
10.1007/s12185-017-2263-8subject
Has Abstractpub_date
2017-09-01 00:00:00pages
357-365issue
3eissn
0925-5710issn
1865-3774pii
10.1007/s12185-017-2263-8journal_volume
106pub_type
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journal_title:International journal of hematology
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journal_title:International journal of hematology
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journal_title:International journal of hematology
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journal_title:International journal of hematology
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journal_title:International journal of hematology
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journal_title:International journal of hematology
pub_type: 杂志文章
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journal_title:International journal of hematology
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journal_title:International journal of hematology
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更新日期:2014-03-01 00:00:00
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journal_title:International journal of hematology
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更新日期:2006-08-01 00:00:00
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journal_title:International journal of hematology
pub_type: 杂志文章
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更新日期:2018-06-01 00:00:00
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pub_type: 杂志文章,评审
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pub_type: 临床试验,杂志文章
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journal_title:International journal of hematology
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journal_title:International journal of hematology
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journal_title:International journal of hematology
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journal_title:International journal of hematology
pub_type: 杂志文章
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更新日期:1994-07-01 00:00:00
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