{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,1,27]],"date-time":"2026-01-27T22:00:31Z","timestamp":1769551231308,"version":"3.49.0"},"reference-count":83,"publisher":"Wiley","issue":"1","license":[{"start":{"date-parts":[[2020,12,9]],"date-time":"2020-12-09T00:00:00Z","timestamp":1607472000000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"funder":[{"DOI":"10.13039\/501100003593","name":"Conselho Nacional de Desenvolvimento Cient\u00edfico e Tecnol\u00f3gico","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100003593","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Int J Experimental Path"],"published-print":{"date-parts":[[2021,2]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>Dystrophin deficiency makes the sarcolemma fragile and susceptible to degeneration in Duchenne muscular dystrophy. The proteasome is a multimeric protease complex and is central to the regulation of cellular proteins. Previous studies have shown that proteasome inhibition improved pathological changes in mdx mice. Ixazomib is the first oral proteasome inhibitor used as a therapy in multiple myeloma. This study investigated the effects of ixazomib on the dystrophic muscle of mdx mice. MDX mice were treated with ixazomib (7.5\u00a0mg\/kg\/wk by gavage) or 0.2\u00a0mL of saline for 12\u00a0weeks. The Kondziela test was performed to measure muscle strength. The tibialis anterior (TA) and diaphragm (DIA) muscles were used for morphological analysis, and blood samples were collected for biochemical measurement. We observed maintenance of the muscle strength in the animals treated with ixazomib. Treatment with ixazomib had no toxic effect on the mdx mouse. The morphological analysis showed a reduction in the inflammatory area and fibres with central nuclei in the TA and DIA muscles and an increase in the number of fibres with a diameter of 20\u00a0\u00b5m<jats:sup>2<\/jats:sup> in the DIA muscle after treatment with ixazomib. There was an increase in the expression of dystrophin and utrophin in the TA and DIA muscles and a reduction in the expression of osteopontin and TGF\u2010\u03b2 in the DIA muscle of mdx mice treated with ixazomib. Ixazomib was thus shown to increase the expression of dystrophin and utrophin associated with improved pathological and functional changes in the dystrophic muscles of mdx mice.<\/jats:p>","DOI":"10.1111\/iep.12383","type":"journal-article","created":{"date-parts":[[2020,12,10]],"date-time":"2020-12-10T16:54:34Z","timestamp":1607619274000},"page":"11-21","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":6,"title":["Ixazomib, an oral proteasome inhibitor, exhibits potential effect in dystrophin\u2010deficient mdx mice"],"prefix":"10.1111","volume":"102","author":[{"given":"Maria Laura Jorge","family":"Micheletto","sequence":"first","affiliation":[{"name":"Departament of Morphology and Physiology Medical Faculty of the ABC Santo Andr\u00e9 Brazil"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-2937-3890","authenticated-orcid":false,"given":"Tulio de Almeida","family":"Hermes","sequence":"additional","affiliation":[{"name":"Departament of Morphology and Physiology Medical Faculty of the ABC Santo Andr\u00e9 Brazil"},{"name":"Departament of Anatomy Federal University of Alfenas Alfenas Brazil"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-7596-0511","authenticated-orcid":false,"given":"Bruno Machado","family":"Bertassoli","sequence":"additional","affiliation":[{"name":"Departament of Morphology and Physiology Medical Faculty of the ABC Santo Andr\u00e9 Brazil"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Giuliana","family":"Petri","sequence":"additional","affiliation":[{"name":"Departament of Morphology and Physiology Medical Faculty of the ABC Santo Andr\u00e9 Brazil"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Matheus Moreira","family":"Perez","sequence":"additional","affiliation":[{"name":"Department of Clinical Analysis Medical Faculty of the ABC Santo Andr\u00e9 Brazil"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Fernando Luiz Affonso","family":"Fonseca","sequence":"additional","affiliation":[{"name":"Department of Clinical Analysis Medical Faculty of the ABC Santo Andr\u00e9 Brazil"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Alzira Alves de Siqueira","family":"Carvalho","sequence":"additional","affiliation":[{"name":"Departament of Morphology and Physiology Medical Faculty of the ABC Santo Andr\u00e9 Brazil"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"David","family":"Feder","sequence":"additional","affiliation":[{"name":"Departament of Morphology and Physiology Medical Faculty of the ABC Santo Andr\u00e9 Brazil"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2020,12,9]]},"reference":[{"key":"e_1_2_9_2_1","doi-asserted-by":"publisher","DOI":"10.1002\/ana.23528"},{"key":"e_1_2_9_3_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0074-7742(08)60083-5"},{"key":"e_1_2_9_4_1","doi-asserted-by":"publisher","DOI":"10.1007\/978-1-4613-0987-1_3"},{"key":"e_1_2_9_5_1","doi-asserted-by":"publisher","DOI":"10.1152\/physrev.00028.2001"},{"key":"e_1_2_9_6_1","doi-asserted-by":"publisher","DOI":"10.1038\/333863a0"},{"key":"e_1_2_9_7_1","doi-asserted-by":"publisher","DOI":"10.1038\/333466a0"},{"key":"e_1_2_9_8_1","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.90.8.3710"},{"key":"e_1_2_9_9_1","doi-asserted-by":"publisher","DOI":"10.1002\/mus.1192"},{"key":"e_1_2_9_10_1","doi-asserted-by":"publisher","DOI":"10.1038\/355696a0"},{"key":"e_1_2_9_11_1","doi-asserted-by":"publisher","DOI":"10.1172\/JCI116989"},{"key":"e_1_2_9_12_1","doi-asserted-by":"publisher","DOI":"10.1097\/00003086-200208000-00012"},{"key":"e_1_2_9_13_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00109-003-0484-1"},{"key":"e_1_2_9_14_1","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.81.4.1189"},{"key":"e_1_2_9_15_1","doi-asserted-by":"publisher","DOI":"10.1007\/BF00687043"},{"key":"e_1_2_9_16_1","doi-asserted-by":"publisher","DOI":"10.1007\/BF00688245"},{"key":"e_1_2_9_17_1","doi-asserted-by":"publisher","DOI":"10.1083\/jcb.151.6.1321"},{"key":"e_1_2_9_18_1","doi-asserted-by":"publisher","DOI":"10.1016\/s0960-8966(02)00242-0"},{"key":"e_1_2_9_19_1","doi-asserted-by":"publisher","DOI":"10.1093\/hmg\/ddz181"},{"key":"e_1_2_9_20_1","doi-asserted-by":"publisher","DOI":"10.1038\/360588a0"},{"key":"e_1_2_9_21_1","doi-asserted-by":"publisher","DOI":"10.1016\/S1074-5521(01)00056-4"},{"key":"e_1_2_9_22_1","doi-asserted-by":"publisher","DOI":"10.1038\/nrd4467"},{"key":"e_1_2_9_23_1","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.1521919112"},{"key":"e_1_2_9_24_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.bcp.2015.04.008"},{"key":"e_1_2_9_25_1","doi-asserted-by":"publisher","DOI":"10.1016\/0006-291X(92)90242-D"},{"key":"e_1_2_9_26_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0002-9440(10)63523-7"},{"key":"e_1_2_9_27_1","doi-asserted-by":"publisher","DOI":"10.4161\/cc.6.10.4182"},{"key":"e_1_2_9_28_1","doi-asserted-by":"publisher","DOI":"10.1038\/mt.2016.162"},{"key":"e_1_2_9_29_1","doi-asserted-by":"publisher","DOI":"10.2353\/ajpath.2010.090468"},{"key":"e_1_2_9_30_1","doi-asserted-by":"publisher","DOI":"10.1007\/s40262-017-0526-4"},{"key":"e_1_2_9_31_1","doi-asserted-by":"publisher","DOI":"10.1158\/0008-5472.CAN-09-2766"},{"key":"e_1_2_9_32_1","doi-asserted-by":"publisher","DOI":"10.1016\/S1470-2045(14)71125-8"},{"key":"e_1_2_9_33_1","doi-asserted-by":"publisher","DOI":"10.1158\/1078-0432.CCR-13-1657"},{"key":"e_1_2_9_34_1","unstructured":"The use of four limb hanging tests to monitor muscle strength and condition over time [Internet].2011."},{"key":"e_1_2_9_35_1","doi-asserted-by":"publisher","DOI":"10.1038\/352536a0"},{"key":"e_1_2_9_36_1","doi-asserted-by":"publisher","DOI":"10.1093\/brain\/110.2.269"},{"key":"e_1_2_9_37_1","unstructured":"Quantitative determination of muscle fiber diameter (minimal Feret\u2019s diameter) and percentage of centralized nuclei[Internet]..2008."},{"key":"e_1_2_9_38_1","doi-asserted-by":"publisher","DOI":"10.1371\/journal.pone.0061367"},{"key":"e_1_2_9_39_1","doi-asserted-by":"publisher","DOI":"10.1152\/ajpcell.00434.2005"},{"key":"e_1_2_9_40_1","doi-asserted-by":"publisher","DOI":"10.1016\/0022-510X(90)90195-S"},{"key":"e_1_2_9_41_1","doi-asserted-by":"publisher","DOI":"10.1016\/0960-8966(93)90109-W"},{"key":"e_1_2_9_42_1","doi-asserted-by":"publisher","DOI":"10.1002\/humu.22758"},{"key":"e_1_2_9_43_1","doi-asserted-by":"publisher","DOI":"10.1007\/s004010000229"},{"key":"e_1_2_9_44_1","doi-asserted-by":"publisher","DOI":"10.1007\/s10974-014-9385-x"},{"key":"e_1_2_9_45_1","doi-asserted-by":"publisher","DOI":"10.3390\/ijms21176429"},{"key":"e_1_2_9_46_1","doi-asserted-by":"publisher","DOI":"10.1093\/hmg\/ddx414"},{"key":"e_1_2_9_47_1","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.1508755112"},{"key":"e_1_2_9_48_1","doi-asserted-by":"publisher","DOI":"10.1016\/0092-8674(94)90527-4"},{"key":"e_1_2_9_49_1","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.1001517107"},{"key":"e_1_2_9_50_1","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.1008818107"},{"key":"e_1_2_9_51_1","doi-asserted-by":"publisher","DOI":"10.3233\/JND-180357"},{"key":"e_1_2_9_52_1","doi-asserted-by":"publisher","DOI":"10.1371\/journal.pgen.1004490"},{"key":"e_1_2_9_53_1","doi-asserted-by":"publisher","DOI":"10.1002\/jcsm.12338"},{"key":"e_1_2_9_54_1","doi-asserted-by":"publisher","DOI":"10.1091\/mbc.01-09-0446"},{"key":"e_1_2_9_55_1","doi-asserted-by":"publisher","DOI":"10.1038\/4033"},{"key":"e_1_2_9_56_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.0959-9673.2004.00405.x"},{"key":"e_1_2_9_57_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2014-01-548941"},{"key":"e_1_2_9_58_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMoa1516282"},{"key":"e_1_2_9_59_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2017-03-771220"},{"key":"e_1_2_9_60_1","doi-asserted-by":"publisher","DOI":"10.1371\/journal.pone.0215590"},{"key":"e_1_2_9_61_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.lfs.2015.10.023"},{"key":"e_1_2_9_62_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1440-1754.2010.01730.x"},{"key":"e_1_2_9_63_1","doi-asserted-by":"publisher","DOI":"10.1155\/2018\/6484610"},{"key":"e_1_2_9_64_1","doi-asserted-by":"publisher","DOI":"10.1038\/mt.2008.120"},{"key":"e_1_2_9_65_1","doi-asserted-by":"publisher","DOI":"10.1152\/ajpregu.00684.2004"},{"key":"e_1_2_9_66_1","doi-asserted-by":"publisher","DOI":"10.1097\/00131402-200103000-00008"},{"key":"e_1_2_9_67_1","doi-asserted-by":"publisher","DOI":"10.1002\/(SICI)1097-4598(199805)21:5<567::AID-MUS2>3.0.CO;2-6"},{"key":"e_1_2_9_68_1","doi-asserted-by":"publisher","DOI":"10.1002\/cbin.11057"},{"key":"e_1_2_9_69_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0140-6736(02)07815-7"},{"key":"e_1_2_9_70_1","doi-asserted-by":"publisher","DOI":"10.1002\/ana.410230511"},{"key":"e_1_2_9_71_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2613.2006.00490.x"},{"key":"e_1_2_9_72_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2559.2011.04051.x"},{"key":"e_1_2_9_73_1","doi-asserted-by":"publisher","DOI":"10.1111\/iep.12142"},{"key":"e_1_2_9_74_1","doi-asserted-by":"publisher","DOI":"10.1083\/jcb.201510086"},{"key":"e_1_2_9_75_1","doi-asserted-by":"publisher","DOI":"10.1002\/mus.25752"},{"key":"e_1_2_9_76_1","doi-asserted-by":"publisher","DOI":"10.1172\/JCI37662"},{"key":"e_1_2_9_77_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.pmr.2011.11.014"},{"key":"e_1_2_9_78_1","doi-asserted-by":"publisher","DOI":"10.1002\/mus.880110802"},{"key":"e_1_2_9_79_1","doi-asserted-by":"publisher","DOI":"10.1002\/ar.21224"},{"key":"e_1_2_9_80_1","doi-asserted-by":"publisher","DOI":"10.1016\/0092-8674(88)90463-1"},{"key":"e_1_2_9_81_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0014-5793(04)00179-6"},{"key":"e_1_2_9_82_1","doi-asserted-by":"publisher","DOI":"10.1152\/japplphysiol.00182.2003"},{"key":"e_1_2_9_83_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2613.2007.00553.x"},{"key":"e_1_2_9_84_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.nbd.2008.03.008"}],"container-title":["International Journal of Experimental Pathology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1111\/iep.12383","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/full-xml\/10.1111\/iep.12383","content-type":"application\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1111\/iep.12383","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,9,3]],"date-time":"2023-09-03T16:43:28Z","timestamp":1693759408000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1111\/iep.12383"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2020,12,9]]},"references-count":83,"journal-issue":{"issue":"1","published-print":{"date-parts":[[2021,2]]}},"alternative-id":["10.1111\/iep.12383"],"URL":"https:\/\/doi.org\/10.1111\/iep.12383","archive":["Portico"],"relation":{},"ISSN":["0959-9673","1365-2613"],"issn-type":[{"value":"0959-9673","type":"print"},{"value":"1365-2613","type":"electronic"}],"subject":[],"published":{"date-parts":[[2020,12,9]]},"assertion":[{"value":"2020-06-29","order":0,"name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2020-11-04","order":1,"name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}},{"value":"2020-12-09","order":2,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}]}}