{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,3,24]],"date-time":"2026-03-24T22:04:26Z","timestamp":1774389866900,"version":"3.50.1"},"reference-count":24,"publisher":"American Society of Hematology","issue":"7","content-domain":{"domain":["ashpublications.org"],"crossmark-restriction":true},"short-container-title":[],"published-print":{"date-parts":[[2004,4,1]]},"abstract":"<jats:title>Abstract<\/jats:title>\n               <jats:p>Genetic causes of hereditary hemochromatosis (HH) include mutations in the HFE gene, coding for a \u03b22-microglobulin (\u03b22m)-associated major histocompatibility complex class I-like protein. However, iron accumulation in patients with HH can be highly variable. Previously, analysis of \u03b22mRag1-\/- double-deficient mice, lacking all \u03b22m-dependent molecules and lymphocytes, demonstrated increased iron accumulation in the pancreas and heart compared with \u03b22m single knock-out mice. To evaluate whether the observed phenotype in \u03b22mRag1-\/- mice was due solely to the absence of Hfe or to other \u03b22m-dependent molecules, we generated HfeRag1-\/- double-deficient mice. Our studies revealed that introduction of Rag1 deficiency in Hfe knock-out mice leads to heightened iron overload, mainly in the liver, whereas the heart and pancreas are relatively spared compared with \u03b22mRag1-\/- mice. These results suggest that other \u03b22m-interacting protein(s) may be involved in iron regulation and that in the absence of functional Hfe molecules lymphocyte numbers may influence iron overload severity. (Blood. 2004;103: 2847-2849)<\/jats:p>","DOI":"10.1182\/blood-2003-09-3300","type":"journal-article","created":{"date-parts":[[2003,12,9]],"date-time":"2003-12-09T01:23:13Z","timestamp":1070932993000},"page":"2847-2849","update-policy":"https:\/\/doi.org\/10.1182\/blood.2019cm0000","source":"Crossref","is-referenced-by-count":27,"title":["Contributions of \u03b22-microglobulin\u2013dependent molecules and lymphocytes to iron regulation: insights from HfeRag1-\/- and \u03b22mRag1-\/- double knock-out mice"],"prefix":"10.1182","volume":"103","author":[{"given":"Carlos J.","family":"Miranda","sequence":"first","affiliation":[{"name":"From the Centre de recherche, Centre Hospitalier de l'Universite\u0301 de Montre\u0301al and the Universite\u0301 de Montre\u0301al, Montreal, QC, Canada; Howard Hughes Medical Institute, Department of Pediatrics, Harvard Medical School, Children's Hospital, Boston, MA; UnIGENe, Instituto de Biologia Molecular e Celular, Porto, Portugal."}]},{"given":"Hortence","family":"Makui","sequence":"additional","affiliation":[{"name":"From the Centre de recherche, Centre Hospitalier de l'Universite\u0301 de Montre\u0301al and the Universite\u0301 de Montre\u0301al, Montreal, QC, Canada; Howard Hughes Medical Institute, Department of Pediatrics, Harvard Medical School, Children's Hospital, Boston, MA; UnIGENe, Instituto de Biologia Molecular e Celular, Porto, Portugal."}]},{"given":"Nancy C.","family":"Andrews","sequence":"additional","affiliation":[{"name":"From the Centre de recherche, Centre Hospitalier de l'Universite\u0301 de Montre\u0301al and the Universite\u0301 de Montre\u0301al, Montreal, QC, Canada; Howard Hughes Medical Institute, Department of Pediatrics, Harvard Medical School, Children's Hospital, Boston, MA; UnIGENe, Instituto de Biologia Molecular e Celular, Porto, Portugal."}]},{"given":"Manuela M.","family":"Santos","sequence":"additional","affiliation":[{"name":"From the Centre de recherche, Centre Hospitalier de l'Universite\u0301 de Montre\u0301al and the Universite\u0301 de Montre\u0301al, Montreal, QC, Canada; Howard Hughes Medical Institute, Department of Pediatrics, Harvard Medical School, Children's Hospital, Boston, MA; UnIGENe, Instituto de Biologia Molecular e Celular, Porto, Portugal."}]}],"member":"234","reference":[{"key":"2020021219262715200_REF1","doi-asserted-by":"crossref","unstructured":"Feder JN, Gnirke A, Thomas W, et al. 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