{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,27]],"date-time":"2026-09-27T19:37:24Z","timestamp":1790537844665,"version":"4.1.0"},"reference-count":33,"publisher":"National Academy of Sciences","issue":"15","content-domain":{"domain":["www.pnas.org"],"crossmark-restriction":true},"short-container-title":["Proc. Natl. Acad. Sci. U.S.A."],"published-print":{"date-parts":[[2002,7,23]]},"abstract":"<jats:p>\n                    Neuronal degeneration in spinal muscular atrophy is caused by reduced expression of the survival motor neuron (SMN) protein. SMN and the tightly interacting Gemin2 form part of a macromolecular complex (SMN complex) that mediates assembly of spliceosomal small nuclear ribonucleoproteins (U snRNPs). We used mouse genetics to investigate the function of this complex in motoneuron maintenance. Reduced Smn\/Gemin2 protein levels lead to disturbed U snRNP assembly as indicated by reduced nuclear accumulation of Sm proteins. This finding correlates with enhanced motoneuron degeneration in\n                    <jats:italic>Gemin2<\/jats:italic>\n                    <jats:sup>+\/\u2212<\/jats:sup>\n                    \/\n                    <jats:italic>Smn<\/jats:italic>\n                    <jats:sup>+\/\u2212<\/jats:sup>\n                    mice. Our data provide\n                    <jats:italic>in vivo<\/jats:italic>\n                    evidence that impaired production of U snRNPs contributes to motoneuron degeneration.\n                  <\/jats:p>","DOI":"10.1073\/pnas.152318699","type":"journal-article","created":{"date-parts":[[2002,7,28]],"date-time":"2002-07-28T18:26:28Z","timestamp":1027880788000},"page":"10126-10131","update-policy":"https:\/\/doi.org\/10.1073\/pnas.cm10313","source":"Crossref","is-referenced-by-count":79,"title":["Gene targeting of Gemin2 in mice reveals a correlation between defects in the biogenesis of U snRNPs and motoneuron cell death"],"prefix":"10.1073","volume":"99","author":[{"given":"Sibylle","family":"Jablonka","sequence":"first","affiliation":[{"name":"Institute of Clinical Neurobiology, Josef-Schneider Strasse 11, D-97080 W\u00fcrzburg, Germany; and Max Planck Institute of Biochemistry, Am Klopferspitz 18a, D-82152 Martinsried, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Bettina","family":"Holtmann","sequence":"additional","affiliation":[{"name":"Institute of Clinical Neurobiology, Josef-Schneider Strasse 11, D-97080 W\u00fcrzburg, Germany; and Max Planck Institute of Biochemistry, Am Klopferspitz 18a, D-82152 Martinsried, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Gunter","family":"Meister","sequence":"additional","affiliation":[{"name":"Institute of Clinical Neurobiology, Josef-Schneider Strasse 11, D-97080 W\u00fcrzburg, Germany; and Max Planck Institute of Biochemistry, Am Klopferspitz 18a, D-82152 Martinsried, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Michael","family":"Bandilla","sequence":"additional","affiliation":[{"name":"Institute of Clinical Neurobiology, Josef-Schneider Strasse 11, D-97080 W\u00fcrzburg, Germany; and Max Planck Institute of Biochemistry, Am Klopferspitz 18a, D-82152 Martinsried, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Wilfried","family":"Rossoll","sequence":"additional","affiliation":[{"name":"Institute of Clinical Neurobiology, Josef-Schneider Strasse 11, D-97080 W\u00fcrzburg, Germany; and Max Planck Institute of Biochemistry, Am Klopferspitz 18a, D-82152 Martinsried, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Utz","family":"Fischer","sequence":"additional","affiliation":[{"name":"Institute of Clinical Neurobiology, Josef-Schneider Strasse 11, D-97080 W\u00fcrzburg, Germany; and Max Planck Institute of Biochemistry, Am Klopferspitz 18a, D-82152 Martinsried, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Michael","family":"Sendtner","sequence":"additional","affiliation":[{"name":"Institute of Clinical Neurobiology, Josef-Schneider Strasse 11, D-97080 W\u00fcrzburg, Germany; and Max Planck Institute of Biochemistry, Am Klopferspitz 18a, D-82152 Martinsried, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"341","published-online":{"date-parts":[[2002,6,28]]},"reference":[{"key":"e_1_3_3_1_2","doi-asserted-by":"publisher","DOI":"10.1212\/WNL.46.2.335"},{"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":"10.1006\/geno.1999.5910"},{"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":"10.1002\/(SICI)1098-1004(200003)15:3<228::AID-HUMU3>3.0.CO;2-9"},{"key":"e_1_3_3_6_2","doi-asserted-by":"publisher","DOI":"10.1007\/s004390100473"},{"key":"e_1_3_3_7_2","doi-asserted-by":"publisher","DOI":"10.1093\/hmg\/9.5.663"},{"key":"e_1_3_3_8_2","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.94.18.9920"},{"key":"e_1_3_3_9_2","doi-asserted-by":"publisher","DOI":"10.1016\/S0014-5793(00)02243-2"},{"key":"e_1_3_3_10_2","doi-asserted-by":"publisher","DOI":"10.1093\/hmg\/8.12.2133"},{"key":"e_1_3_3_11_2","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.M001441200"},{"key":"e_1_3_3_12_2","doi-asserted-by":"publisher","DOI":"10.1093\/hmg\/9.3.341"},{"key":"e_1_3_3_13_2","doi-asserted-by":"publisher","DOI":"10.1093\/hmg\/9.3.333"},{"key":"e_1_3_3_14_2","doi-asserted-by":"publisher","DOI":"10.1016\/S0092-8674(00)80367-0"},{"key":"e_1_3_3_15_2","doi-asserted-by":"publisher","DOI":"10.1002\/j.1460-2075.1996.tb00725.x"},{"key":"e_1_3_3_16_2","doi-asserted-by":"publisher","DOI":"10.1093\/hmg\/9.13.1977"},{"key":"e_1_3_3_17_2","doi-asserted-by":"publisher","DOI":"10.1093\/hmg\/9.7.1093"},{"key":"e_1_3_3_18_2","doi-asserted-by":"publisher","DOI":"10.1083\/jcb.147.6.1181"},{"key":"e_1_3_3_19_2","doi-asserted-by":"publisher","DOI":"10.1128\/JVI.75.23.11781-11790.2001"},{"key":"e_1_3_3_20_2","doi-asserted-by":"publisher","DOI":"10.1083\/jcb.148.6.1177"},{"key":"e_1_3_3_21_2","doi-asserted-by":"publisher","DOI":"10.1038\/ncb1101-945"},{"key":"e_1_3_3_22_2","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.M109448200"},{"key":"e_1_3_3_23_2","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.M110141200"},{"key":"e_1_3_3_24_2","doi-asserted-by":"publisher","DOI":"10.1093\/hmg\/8.13.2351"},{"key":"e_1_3_3_25_2","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.96.20.11167"},{"key":"e_1_3_3_26_2","doi-asserted-by":"publisher","DOI":"10.1016\/S0092-8674(00)80368-2"},{"key":"e_1_3_3_27_2","doi-asserted-by":"publisher","DOI":"10.1016\/S0092-8674(00)81632-3"},{"key":"e_1_3_3_28_2","doi-asserted-by":"publisher","DOI":"10.1016\/S0955-0674(00)00211-8"},{"key":"e_1_3_3_29_2","doi-asserted-by":"publisher","DOI":"10.1038\/sj.ejhg.5200479"},{"key":"e_1_3_3_30_2","doi-asserted-by":"publisher","DOI":"10.1093\/hmg\/10.5.497"},{"key":"e_1_3_3_31_2","doi-asserted-by":"publisher","DOI":"10.1016\/0076-6879(93)25030-6"},{"key":"e_1_3_3_32_2","doi-asserted-by":"publisher","DOI":"10.1002\/art.1780250709"},{"key":"e_1_3_3_33_2","doi-asserted-by":"publisher","DOI":"10.1038\/365027a0"}],"container-title":["Proceedings of the National Academy of Sciences"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/pnas.org\/doi\/pdf\/10.1073\/pnas.152318699","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2022,4,13]],"date-time":"2022-04-13T01:33:52Z","timestamp":1649813632000},"score":1,"resource":{"primary":{"URL":"https:\/\/pnas.org\/doi\/full\/10.1073\/pnas.152318699"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2002,6,28]]},"references-count":33,"journal-issue":{"issue":"15","published-print":{"date-parts":[[2002,7,23]]}},"alternative-id":["10.1073\/pnas.152318699"],"URL":"https:\/\/doi.org\/10.1073\/pnas.152318699","relation":{},"ISSN":["0027-8424","1091-6490"],"issn-type":[{"value":"0027-8424","type":"print"},{"value":"1091-6490","type":"electronic"}],"subject":[],"published":{"date-parts":[[2002,6,28]]},"assertion":[{"value":"2002-04-04","order":0,"name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2002-05-28","order":1,"name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}},{"value":"2002-06-28","order":2,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}]}}