{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,17]],"date-time":"2026-10-17T20:05:43Z","timestamp":1792267543607,"version":"4.4.0"},"reference-count":27,"publisher":"National Academy of Sciences","issue":"12","content-domain":{"domain":["www.pnas.org"],"crossmark-restriction":true},"short-container-title":["Proc. Natl. Acad. Sci. U.S.A."],"published-print":{"date-parts":[[2001,6,5]]},"abstract":"<jats:p>\n                    Copper plays a fundamental role in the biochemistry of all aerobic\n organisms. The delivery of this metal to specific intracellular targets\n is mediated by metallochaperones. To elucidate the role of the\n metallochaperone Atox1, we analyzed mice with a disruption of\n the\n                    <jats:italic>Atox1<\/jats:italic>\n                    locus.\n                    <jats:italic>Atox1<\/jats:italic>\n                    <jats:sup>\u2212\/\u2212<\/jats:sup>\n                    mice failed to thrive immediately after birth, with 45% of pups dying\n before weaning. Surviving animals exhibited growth failure, skin\n laxity, hypopigmentation, and seizures because of perinatal copper\n deficiency. Maternal Atox1 deficiency markedly increased the severity\n of\n                    <jats:italic>Atox1<\/jats:italic>\n                    <jats:sup>\u2212\/\u2212<\/jats:sup>\n                    phenotype, resulting in\n increased perinatal mortality as well as severe growth retardation and\n congenital malformations among surviving\n                    <jats:italic>Atox1<\/jats:italic>\n                    <jats:sup>\u2212\/\u2212<\/jats:sup>\n                    progeny. Furthermore,\n Atox1-deficient cells accumulated high levels of intracellular copper,\n and metabolic studies indicated that this defect was because of\n impaired cellular copper efflux. Taken together, these data reveal a\n direct role for Atox1 in trafficking of intracellular copper to the\n secretory pathway of mammalian cells and demonstrate that this\n metallochaperone plays a critical role in perinatal copper\n homeostasis.\n                  <\/jats:p>","DOI":"10.1073\/pnas.111058498","type":"journal-article","created":{"date-parts":[[2002,7,26]],"date-time":"2002-07-26T10:37:36Z","timestamp":1027679856000},"page":"6848-6852","update-policy":"https:\/\/doi.org\/10.1073\/pnas.cm10313","source":"Crossref","is-referenced-by-count":219,"title":["The metallochaperone Atox1 plays a critical role in perinatal copper homeostasis"],"prefix":"10.1073","volume":"98","author":[{"given":"Iqbal","family":"Hamza","sequence":"first","affiliation":[{"name":"Department of Pediatrics, Washington University School of\r Medicine, St. Louis, MO 63110; Department of Molecular\r Cell Biology, Max Planck Institute, 37077 Gottingen, Germany; and\r Department of Biochemistry and Molecular Biology,\r University of Minnesota Duluth School of Medicine, Duluth, MN 55812"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Anja","family":"Faisst","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Washington University School of\r Medicine, St. Louis, MO 63110; Department of Molecular\r Cell Biology, Max Planck Institute, 37077 Gottingen, Germany; and\r Department of Biochemistry and Molecular Biology,\r University of Minnesota Duluth School of Medicine, Duluth, MN 55812"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Joseph","family":"Prohaska","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Washington University School of\r Medicine, St. Louis, MO 63110; Department of Molecular\r Cell Biology, Max Planck Institute, 37077 Gottingen, Germany; and\r Department of Biochemistry and Molecular Biology,\r University of Minnesota Duluth School of Medicine, Duluth, MN 55812"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Joseph","family":"Chen","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Washington University School of\r Medicine, St. Louis, MO 63110; Department of Molecular\r Cell Biology, Max Planck Institute, 37077 Gottingen, Germany; and\r Department of Biochemistry and Molecular Biology,\r University of Minnesota Duluth School of Medicine, Duluth, MN 55812"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Peter","family":"Gruss","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Washington University School of\r Medicine, St. Louis, MO 63110; Department of Molecular\r Cell Biology, Max Planck Institute, 37077 Gottingen, Germany; and\r Department of Biochemistry and Molecular Biology,\r University of Minnesota Duluth School of Medicine, Duluth, MN 55812"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jonathan D.","family":"Gitlin","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Washington University School of\r Medicine, St. Louis, MO 63110; Department of Molecular\r Cell Biology, Max Planck Institute, 37077 Gottingen, Germany; and\r Department of Biochemistry and Molecular Biology,\r University of Minnesota Duluth School of Medicine, Duluth, MN 55812"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"341","published-online":{"date-parts":[[2001,6,5]]},"reference":[{"key":"e_1_3_3_1_2","doi-asserted-by":"publisher","DOI":"10.1093\/jn\/129.7.1251"},{"key":"e_1_3_3_2_2","first-page":"3105","volume-title":"The Metabolic and Molecular Bases of Inherited Diseases","author":"Culotta V C","year":"2001","unstructured":"V C Culotta, J D Gitlin The Metabolic and Molecular Bases of Inherited Diseases, eds C R Scriver, A L Beaudet, W S Sly, D Valle (McGraw\u2013Hill, New York) 2, 3105\u20133126 (2001)."},{"key":"e_1_3_3_3_2","doi-asserted-by":"publisher","DOI":"10.1126\/science.278.5339.817"},{"key":"e_1_3_3_4_2","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.R000006200"},{"key":"e_1_3_3_5_2","doi-asserted-by":"publisher","DOI":"10.1126\/science.284.5415.805"},{"key":"e_1_3_3_6_2","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.272.14.9215"},{"key":"e_1_3_3_7_2","doi-asserted-by":"publisher","DOI":"10.1126\/science.278.5339.853"},{"key":"e_1_3_3_8_2","doi-asserted-by":"publisher","DOI":"10.1016\/S0969-2126(99)80082-3"},{"key":"e_1_3_3_9_2","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.274.40.28497"},{"key":"e_1_3_3_10_2","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.96.23.13363"},{"key":"e_1_3_3_11_2","doi-asserted-by":"publisher","DOI":"10.1002\/(SICI)1097-0177(199806)212:2<293::AID-AJA14>3.0.CO;2-5"},{"key":"e_1_3_3_12_2","doi-asserted-by":"publisher","DOI":"10.1006\/geno.1999.6046"},{"key":"e_1_3_3_13_2","doi-asserted-by":"publisher","DOI":"10.1038\/188150a0"},{"key":"e_1_3_3_14_2","doi-asserted-by":"publisher","DOI":"10.1042\/bj1860629"},{"key":"e_1_3_3_15_2","volume-title":"Cells: A Laboratory Manual","author":"Spector D L","year":"1998","unstructured":"D L Spector, R D Goldman, L A Leinwand Cells: A Laboratory Manual (Cold Spring Harbor Lab. 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