{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,5]],"date-time":"2026-10-05T03:09:42Z","timestamp":1791169782433,"version":"4.1.0"},"reference-count":28,"publisher":"National Academy of Sciences","issue":"19","content-domain":{"domain":["www.pnas.org"],"crossmark-restriction":true},"short-container-title":["Proc. Natl. Acad. Sci. U.S.A."],"published-print":{"date-parts":[[2006,5,9]]},"abstract":"<jats:p>\n                    Mutations that cause reduced expression of the full-length Survival Motor Neurons (SMN) protein are a major cause of spinal muscular atrophy (SMA), a disease characterized by degeneration of the \u03b1-motor neurons in the anterior horn of the spinal cord. The severity of SMA may be influenced by the actions of modifier genes. One potential modifier gene is represented by\n                    <jats:italic>ZPR1<\/jats:italic>\n                    , which is down-regulated in patients with SMA and encodes a zinc finger protein that interacts with complexes formed by SMN. To test the functional significance of\n                    <jats:italic>ZPR1<\/jats:italic>\n                    gene down-regulation, we examined a mouse model with targeted ablation of the\n                    <jats:italic>Zpr1<\/jats:italic>\n                    gene. We report that ZPR1-deficient mice exhibit axonal pathology and neurodegeneration. These data identify ZPR1 deficiency as a contributing factor in neurodegenerative disorders.\n                  <\/jats:p>","DOI":"10.1073\/pnas.0602057103","type":"journal-article","created":{"date-parts":[[2006,4,28]],"date-time":"2006-04-28T20:39:51Z","timestamp":1146256791000},"page":"7471-7475","update-policy":"https:\/\/doi.org\/10.1073\/pnas.cm10313","source":"Crossref","is-referenced-by-count":48,"title":["Deficiency of the zinc finger protein ZPR1 causes neurodegeneration"],"prefix":"10.1073","volume":"103","author":[{"given":"Beth","family":"Doran","sequence":"first","affiliation":[{"name":"*Program in Molecular Medicine,"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Norberto","family":"Gherbesi","sequence":"additional","affiliation":[{"name":"Department of Cell Biology, and"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Gregory","family":"Hendricks","sequence":"additional","affiliation":[{"name":"Department of Cell Biology, and"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Richard A.","family":"Flavell","sequence":"additional","affiliation":[{"name":"Howard Hughes Medical Institute and Section of Immunobiology, Yale University School of Medicine, New Haven, CT 06520"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Roger J.","family":"Davis","sequence":"additional","affiliation":[{"name":"*Program in Molecular Medicine,"},{"name":"Howard Hughes Medical Institute, University of Massachusetts Medical School, Worcester, MA 01605; and"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Laxman","family":"Gangwani","sequence":"additional","affiliation":[{"name":"*Program in Molecular Medicine,"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"341","published-online":{"date-parts":[[2006,5,9]]},"reference":[{"key":"e_1_3_3_1_2","doi-asserted-by":"publisher","DOI":"10.1177\/0884217503261125"},{"key":"e_1_3_3_2_2","doi-asserted-by":"publisher","DOI":"10.1016\/0092-8674(95)90460-3"},{"key":"e_1_3_3_3_2","doi-asserted-by":"publisher","DOI":"10.1038\/ng0797-265"},{"key":"e_1_3_3_4_2","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.96.11.6307"},{"key":"e_1_3_3_5_2","doi-asserted-by":"publisher","DOI":"10.1038\/35070059"},{"key":"e_1_3_3_6_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.yexcr.2004.03.022"},{"key":"e_1_3_3_7_2","doi-asserted-by":"publisher","DOI":"10.1016\/S0960-8966(97)00459-8"},{"key":"e_1_3_3_8_2","doi-asserted-by":"publisher","DOI":"10.1007\/s100480050021"},{"key":"e_1_3_3_9_2","doi-asserted-by":"publisher","DOI":"10.1007\/s00439-003-1025-2"},{"key":"e_1_3_3_10_2","doi-asserted-by":"publisher","DOI":"10.1016\/0092-8674(95)90461-1"},{"key":"e_1_3_3_11_2","first-page":"72","volume":"60","author":"Burglen L.","year":"1997","unstructured":"L. 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