Disease: Severe combined immunodeficiency with sensitivity to ionizing radiation
- A human severe combined immunodeficiency (SCID) condition with increased sensitivity to ionizing radiations and impaired V(D)J rearrangements defines a new DNA recombination/repair deficiency
- A new type of radiosensitive T-B-NK+ severe combined immunodeficiency caused by a LIG4 mutation
- A novel missense RAG-1 mutation results in T-B-NK+ SCID in Athabascan-speaking Dine Indians from the Canadian Northwest Territories
- A novel radiosensitive SCID patient with a pronounced G(2)/M sensitivity
- A targeted DNA-PKcs-null mutation reveals DNA-PK-independent functions for KU in V(D)J recombination
- An ionizing radiation-sensitive CHO mutant cell line: irs-20. IV. Genetic complementation, V(D)J recombination and the scid phenotype
- Analysis of the defect in DNA end joining in the murine scid mutation
- Artemis deficiency confers a DNA double-strand break repair defect and Artemis phosphorylation status is altered by DNA damage and cell cycle progression
- Artemis splice defects cause atypical SCID and can be restored in vitro by an antisense oligonucleotide
- Bowman-Birk protease inhibitor activates DNA-dependent protein kinase and reduces formation of radiation-induced dicentric chromosomes
- Defective DNA repair and increased genomic instability in Artemis-deficient murine cells
- Defective repair of radiation-induced chromosomal damage in scid/scid mice
- Deletion of Ku70, Ku80, or both causes early aging without substantially increased cancer
- Direct evidence that apoptosis enhances tumor responses to fractionated radiotherapy
- DNA Double Strand Break Response and Limited Repair Capacity in Mouse Elongated Spermatids
- DNA ligase IV syndrome; a review
- DNA-PKcs: a T-cell tumour suppressor encoded at the mouse scid locus
- Dose rate effectiveness and potentially lethal damage repair in normal and double-strand break repair deficient murine cells by gamma-rays and 5-fluorouracil
- Extreme Phenotypes With Identical Mutations: Two Patients With Same Non-sense <em>NHEJ1</em> Homozygous Mutation
- Hemopoietic stem-cell compartment of the SCID mouse: double-exponential survival curve after gamma irradiation
- Homozygous DNA ligase IV R278H mutation in mice leads to leaky SCID and represents a model for human LIG4 syndrome
- Impact of stromal sensitivity on radiation response of tumors implanted in SCID hosts revisited
- Lack of detectable defect in DNA double-strand break repair and DNA-dependent protein kinase activity in radiosensitive human severe combined immunodeficiency fibroblasts
- Leaky Scid phenotype associated with defective V(D)J coding end processing in Artemis-deficient mice
- Lentivirus Mediated Correction of Artemis-Deficient Severe Combined Immunodeficiency
- Nijmegen breakage syndrome (NBS)
- Not All SCID Pigs Are Created Equally: Two Independent Mutations in the Artemis Gene Cause SCID in Pigs
- Novel <em>NHEJ1</em> pathogenic variant linked to severe combined immunodeficiency, microcephaly, and abnormal T and B cell receptor repertoires
- Novel compound heterozygous DNA ligase IV mutations in an adolescent with a slowly-progressing radiosensitive-severe combined immunodeficiency
- p16 gene therapy: a potentially efficacious modality for nasopharyngeal carcinoma
- Radiation-sensitive severe combined immunodeficiency: The arguments for and against conditioning before hematopoietic cell transplantation--what to do?
- Radiosensitive SCID patients with Artemis gene mutations show a complete B-cell differentiation arrest at the pre-B-cell receptor checkpoint in bone marrow
- Radiosensitive severe combined immunodeficiency disease
- RAG1/2 knockout pigs with severe combined immunodeficiency
- Role for Artemis nuclease in the repair of radiation-induced DNA double strand breaks by alternative end joining
- Role of Artemis in DSB repair and guarding chromosomal stability following exposure to ionizing radiation at different stages of cell cycle
- scid cells are deficient in Ku and replication protein A phosphorylation by the DNA-dependent protein kinase
- Severe combined immunodeficiency and microcephaly in siblings with hypomorphic mutations in DNA ligase IV
- Structural analysis of the basal state of the Artemis:DNA-PKcs complex
- Structural and mechanistic insights into the Artemis endonuclease and strategies for its inhibition
- Successful TCRαβ/CD19-Depleted Hematopoietic Cell Transplantation for a Patient With Artemis Deficiency
- Targeted disruption of the Artemis murine counterpart results in SCID and defective V(D)J recombination that is partially corrected with bone marrow transplantation
- The clinical impact of deficiency in DNA non-homologous end-joining
- The neoplastic transformation of SCID cells by radiation
- Unexpected effects of the severe combined immunodeficiency mutation on murine lymphomagenesis