{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,3,16]],"date-time":"2026-03-16T15:00:11Z","timestamp":1773673211727,"version":"3.50.1"},"reference-count":42,"publisher":"Oxford University Press (OUP)","issue":"2","license":[{"start":{"date-parts":[[2020,2,9]],"date-time":"2020-02-09T00:00:00Z","timestamp":1581206400000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/academic.oup.com\/journals\/pages\/open_access\/funder_policies\/chorus\/standard_publication_model"}],"funder":[{"name":"The Campbell Chair for Immunology Research"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2020,4,16]]},"abstract":"<jats:title>Summary<\/jats:title>\n               <jats:p>The absence of adenosine deaminase (ADA) causes severe combined immune deficiency (SCID), which has been treated with PEGylated bovine-extracted ADA (ADAGEN). ADAGEN was recently replaced by a PEGylated recombinant bovine ADA, expressed in Escherichia coli (elapegademase, ELA\u2013ADA). Limited information on ELA\u2013ADA is available. \u00a0ADA enzymatic activity of ELA\u2013ADA and ADAGEN was assessed in vitro at diverse dilutions. ADA activity and immune reconstitution in an ADA\u2013SCID patient treated with ELA\u2013ADA were compared with age-matched patients previously treated with ADAGEN. ADA activity and thymus reconstitution were evaluated in ADA-deficient mice following ELA\u2013ADA or ADAGEN administered from 7\u00a0days postpartum. In vitro, ADA activity of ELA\u2013ADA and ADAGEN were similar at all dilutions. In an ADA\u2013SCID patient, ELA\u2013ADA treatment led to a marked increase in trough plasma ADA activity, which was 20% higher than in a patient previously treated with ADAGEN. A marked increase in T cell numbers and generation of naive T cells was evident following 3\u00a0months of ELA\u2013ADA treatment, while T cell numbers increased following 4 months in 3 patients previously treated with ADAGEN. T cell proliferations stimulation normalized and thymus shadow became evident following ELA\u2013ADA treatment. ADA activity was significantly increased in the blood of ADA-deficient mice following ELA\u2013ADA compared to ADAGEN, while both treatments improved the mice weights, the weight, number of cells in their thymus and thymocyte subpopulations. ELA\u2013ADA has similar in- vitro and possibly better in-vivo activity than ADAGEN. Future studies will determine whether ELA\u2013ADA results in improved long-term immune reconstitution.<\/jats:p>","DOI":"10.1111\/cei.13420","type":"journal-article","created":{"date-parts":[[2020,1,28]],"date-time":"2020-01-28T08:22:26Z","timestamp":1580199746000},"page":"176-184","source":"Crossref","is-referenced-by-count":27,"title":["Comparison of elapegademase and pegademase in ADA-deficient patients and mice"],"prefix":"10.1093","volume":"200","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-6893-3366","authenticated-orcid":false,"given":"L","family":"Murguia-Favela","sequence":"first","affiliation":[{"name":"Section of Hematology and Immunology, Department of Pediatrics, Alberta Children's Hospital and University of Calgary, Calgary, Canada"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"W","family":"Min","sequence":"additional","affiliation":[{"name":"Developmental and Stem Cell Biology Program, Research Institute, Hospital for Sick Children, Toronto, Canada"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"R","family":"Loves","sequence":"additional","affiliation":[{"name":"Developmental and Stem Cell Biology Program, Research Institute, Hospital for Sick Children, Toronto, Canada"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"M","family":"Leon-Ponte","sequence":"additional","affiliation":[{"name":"Developmental and Stem Cell Biology Program, Research Institute, Hospital for Sick Children, Toronto, Canada"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-2586-6391","authenticated-orcid":false,"given":"E","family":"Grunebaum","sequence":"additional","affiliation":[{"name":"Developmental and Stem Cell Biology Program, Research Institute, Hospital for Sick Children, Toronto, Canada"},{"name":"Division of Immunology and Allergy, Department of Pediatrics, Hospital for Sick Children, Toronto, 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