{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,5,2]],"date-time":"2026-05-02T01:56:04Z","timestamp":1777686964270,"version":"3.51.4"},"reference-count":50,"publisher":"SAGE Publications","issue":"1","license":[{"start":{"date-parts":[[2025,10,28]],"date-time":"2025-10-28T00:00:00Z","timestamp":1761609600000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"},{"start":{"date-parts":[[2025,10,28]],"date-time":"2025-10-28T00:00:00Z","timestamp":1761609600000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/journals.sagepub.com\/page\/policies\/text-and-data-mining-license"}],"funder":[{"DOI":"10.13039\/501100000024","name":"Canadian Institute of Health Research","doi-asserted-by":"crossref","award":["272311"],"award-info":[{"award-number":["272311"]}],"id":[{"id":"10.13039\/501100000024","id-type":"DOI","asserted-by":"crossref"}]},{"name":"Fonds de Recherche du Qu\u00e9bec en Sant\u00e9"},{"DOI":"10.13039\/501100000268","name":"Biotechnology and Biological Sciences Research Council","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100000268","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["journals.sagepub.com"],"crossmark-restriction":true},"short-container-title":["Journal of Huntington's Disease"],"published-print":{"date-parts":[[2026,2]]},"abstract":"<jats:p>\n                    <jats:bold>Background<\/jats:bold>\n                    : Huntington's disease (HD) is a progressive neurodegenerative disorder primarily affecting the central nervous system (CNS). However, emerging evidence suggests that peripheral tissues, including skeletal muscle and bone, also undergo pathological changes contributing to disease burden.\n                    <jats:bold>Objective<\/jats:bold>\n                    : To characterize musculoskeletal impairments in the zQ175 knock-in (KI) mouse model of HD, through integrated behavioral, biomechanical, and imaging analyses.\n                    <jats:bold>Methods<\/jats:bold>\n                    : Motor function was assessed using grip strength, rotarod, and open field testing.\n                    <jats:italic toggle=\"yes\">Ex vivo<\/jats:italic>\n                    contractility of the extensor digitorum longus (EDL) and Soleus (Sol) muscles was measured. Muscle fiber cross-sectional area (CSA) was quantified using semi-automated segmentation. Bone microarchitecture was analyzed using high-resolution micro-computed tomography (\u03bcCT).\n                    <jats:bold>Results<\/jats:bold>\n                    : Six-month-old homozygous zQ175 mice exhibited significantly reduced muscle strength and impaired contractile properties in both the EDL and Soleus muscles compared to wild-type (WT) controls. \u00b5CT analysis revealed decreased trabecular bone volume and alterations in bone structure.\n                    <jats:bold>Conclusions<\/jats:bold>\n                    : These findings provide a comprehensive musculoskeletal phenotyping of zQ175 mice, revealing early-onset muscle atrophy and skeletal fragility. Our study highlights the importance of targeting peripheral manifestations in HD and establishes zQ175 KI mice as a valuable additional model for investigating systemic disease mechanisms.\n                  <\/jats:p>","DOI":"10.1177\/18796397251387208","type":"journal-article","created":{"date-parts":[[2025,10,28]],"date-time":"2025-10-28T15:36:13Z","timestamp":1761665773000},"page":"95-109","update-policy":"https:\/\/doi.org\/10.1177\/sage-journals-update-policy","source":"Crossref","is-referenced-by-count":0,"title":["Morphological and functional phenotyping of skeletal muscle and bone in the zQ175 knock-in mouse model of Huntington's disease"],"prefix":"10.1177","volume":"15","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-9790-6658","authenticated-orcid":false,"given":"Behnaz","family":"Nateghi","sequence":"first","affiliation":[{"name":"Centre de Recherche du CHU de Qu\u00e9bec-Universit\u00e9 Laval, CHUL"},{"name":"Facult\u00e9 de M\u00e9decine, D\u00e9partement de Psychiatrie et de Neurosciences, Universit\u00e9 Laval, Qu\u00e9bec, Canada"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-4995-5604","authenticated-orcid":false,"given":"Mohamed","family":"Lala Bouali","sequence":"additional","affiliation":[{"name":"Centre de Recherche du CHU de Qu\u00e9bec-Universit\u00e9 Laval, CHUL"},{"name":"Facult\u00e9 de M\u00e9decine, D\u00e9partement de Psychiatrie et de Neurosciences, Universit\u00e9 Laval, Qu\u00e9bec, Canada"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Zineb","family":"Bouredji","sequence":"additional","affiliation":[{"name":"Centre de Recherche du CHU de Qu\u00e9bec-Universit\u00e9 Laval, CHUL"},{"name":"Facult\u00e9 de M\u00e9decine, D\u00e9partement de Psychiatrie et de Neurosciences, Universit\u00e9 Laval, Qu\u00e9bec, Canada"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Anteneh","family":"Argaw","sequence":"additional","affiliation":[{"name":"Centre de Recherche du CHU de Qu\u00e9bec-Universit\u00e9 Laval, CHUL"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Soher Nagi","family":"Jayash","sequence":"additional","affiliation":[{"name":"University of Edinburgh, Easter Bush"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Colin","family":"Farquharson","sequence":"additional","affiliation":[{"name":"University of Edinburgh, Easter Bush"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"J\u00e9r\u00f4me","family":"Frenette","sequence":"additional","affiliation":[{"name":"Centre de Recherche du CHU de Qu\u00e9bec-Universit\u00e9 Laval, CHUL"},{"name":"\u00c9cole des Sciences de la R\u00e9adaptation, Facult\u00e9 de M\u00e9decine, Universit\u00e9 Laval, Qu\u00e9bec City, Canada"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-0391-9325","authenticated-orcid":false,"given":"S\u00e9bastien S","family":"H\u00e9bert","sequence":"additional","affiliation":[{"name":"Centre de Recherche du CHU de Qu\u00e9bec-Universit\u00e9 Laval, CHUL"},{"name":"Facult\u00e9 de M\u00e9decine, D\u00e9partement de Psychiatrie et de Neurosciences, Universit\u00e9 Laval, Qu\u00e9bec, Canada"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"179","published-online":{"date-parts":[[2025,10,28]]},"reference":[{"key":"e_1_3_3_2_2","doi-asserted-by":"publisher","DOI":"10.1038\/nrdp.2015.5"},{"key":"e_1_3_3_3_2","doi-asserted-by":"publisher","DOI":"10.1038\/nrg1686"},{"key":"e_1_3_3_4_2","doi-asserted-by":"publisher","DOI":"10.1111\/j.1460-9568.2004.03783.x"},{"key":"e_1_3_3_5_2","doi-asserted-by":"publisher","DOI":"10.1038\/sj.cdd.4401967"},{"key":"e_1_3_3_6_2","doi-asserted-by":"publisher","DOI":"10.3390\/cells11172662"},{"key":"e_1_3_3_7_2","doi-asserted-by":"publisher","DOI":"10.3390\/ijms22168363"},{"key":"e_1_3_3_8_2","doi-asserted-by":"publisher","DOI":"10.1016\/0896-6273(93)90127-D"},{"key":"e_1_3_3_9_2","doi-asserted-by":"publisher","DOI":"10.3390\/jfmk7020040"},{"key":"e_1_3_3_10_2","doi-asserted-by":"publisher","DOI":"10.1038\/s41598-017-14290-3"},{"key":"e_1_3_3_11_2","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.M506288200"},{"key":"e_1_3_3_12_2","doi-asserted-by":"publisher","DOI":"10.1093\/hmg\/ddr541"},{"key":"e_1_3_3_13_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.nut.2018.08.005"},{"key":"e_1_3_3_14_2","doi-asserted-by":"publisher","DOI":"10.1371\/journal.pone.0146480"},{"key":"e_1_3_3_15_2","doi-asserted-by":"publisher","DOI":"10.1152\/ajpcell.00285.2015"},{"key":"e_1_3_3_16_2","article-title":"Physiological role of receptor activator nuclear factor-kB (RANK) in denervation-induced muscle atrophy and dysfunction","volume":"3","author":"Dufresne SS","year":"2016","unstructured":"Dufresne SS, Boulanger-Piette A, Boss\u00e9 S, et al. 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