{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,2]],"date-time":"2026-10-02T10:38:05Z","timestamp":1790937485164,"version":"4.1.0"},"reference-count":26,"publisher":"Oxford University Press (OUP)","issue":"4","license":[{"start":{"date-parts":[[2005,2,1]],"date-time":"2005-02-01T00:00:00Z","timestamp":1107216000000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/academic.oup.com\/pages\/standard-publication-reuse-rights"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2005,2,15]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:p>Artemis is a mammalian protein, the absence of which results in SCID in Athabascan-speaking Native Americans (SCIDA). This novel protein has been implicated in DNA double-strand break repair and V(D)J recombination. We have cloned the Artemis murine counterpart, mArt, and generated a mouse with a targeted disruption of mArt. Artemis-deficient mice show a similar T\u2212B\u2212 NK+ immunodeficiency phenotype, and carry a profound impairment in coding joint rearrangement, while retaining intact signal ends and close to normal signal joint formation. mArt\u2212\/\u2212 embryonic fibroblasts show increased sensitivity to ionizing radiation. Hemopoietic stem cell (HSC) transplantation using 500-5000 enriched congenic, but not allogeneic mismatched HSC corrected the T cell and partially corrected the B cell defect. Large numbers (40,000) of allogeneic mismatched HSC or pretreatment with 300 cGy of radiation overcame graft resistance, resulting in limited B cell engraftment. Our results suggest that the V(D)J and DNA repair defects seen in this mArt\u2212\/\u2212 mouse model are comparable to those in humans with Artemis deficiency, and that the recovery of immunity following HSC transplantation favors T rather than B cell reconstitution, consistent with what is seen in children with this form of SCID.<\/jats:p>","DOI":"10.4049\/jimmunol.174.4.2420","type":"journal-article","created":{"date-parts":[[2014,4,20]],"date-time":"2014-04-20T22:28:51Z","timestamp":1398032931000},"page":"2420-2428","source":"Crossref","is-referenced-by-count":37,"title":["Targeted Disruption of the Artemis Murine Counterpart Results in SCID and Defective V(D)J Recombination That Is Partially Corrected with Bone Marrow Transplantation"],"prefix":"10.1093","volume":"174","author":[{"given":"Lanying","family":"Li","sequence":"first","affiliation":[{"name":"Department of Pediatrics, University of California , San Francisco, CA 94143"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Eduardo","family":"Salido","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, University of California , San Francisco, CA 94143"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yungui","family":"Zhou","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, University of California , San Francisco, CA 94143"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Swati","family":"Bhattacharyya","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, University of California , San Francisco, CA 94143"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Steven M","family":"Yannone","sequence":"additional","affiliation":[{"name":"Department of Molecular Biology, Lawrence Berkeley National Laboratory , Berkeley, CA 94720"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Elizabeth","family":"Dunn","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, University of California , San Francisco, CA 94143"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Juanito","family":"Meneses","sequence":"additional","affiliation":[{"name":"Department of Obstetrics\/Gynecology and Reproductive Science, University of California , San Francisco, CA 94143"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ann J","family":"Feeney","sequence":"additional","affiliation":[{"name":"Department of Immunology, The Scripps Research Institutes , La Jolla, CA 92037"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Morton J","family":"Cowan","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, University of California , San Francisco, CA 94143"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"286","published-online":{"date-parts":[[2005,2,15]]},"reference":[{"key":"2026020212231508000_R1","first-page":"172","volume-title":"Curr. 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