{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,3]],"date-time":"2026-09-03T17:15:13Z","timestamp":1788455713992,"version":"build-2803163510"},"reference-count":57,"publisher":"SAGE Publications","issue":"5","license":[{"start":{"date-parts":[[2026,3,10]],"date-time":"2026-03-10T00:00:00Z","timestamp":1773100800000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/journals.sagepub.com\/page\/policies\/text-and-data-mining-license"}],"funder":[{"name":"JSPS KAKENHI","award":["20K06461"],"award-info":[{"award-number":["20K06461"]}]},{"name":"JSPS KAKENHI","award":["24K09300"],"award-info":[{"award-number":["24K09300"]}]},{"name":"KAC 45th Anniversary Grant"},{"DOI":"10.13039\/501100009583","name":"Morinaga Foundation For Health and Nutrition","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100009583","id-type":"DOI","asserted-by":"publisher"}]},{"name":"Japan Science and Technology Agency (JST) SPRING","award":["JPMJSP2139"],"award-info":[{"award-number":["JPMJSP2139"]}]}],"content-domain":{"domain":["journals.sagepub.com"],"crossmark-restriction":true},"short-container-title":["Vet Pathol"],"published-print":{"date-parts":[[2026,9]]},"abstract":"<jats:p>\n                    Neuroaxonal dystrophy is a group of genetic neurodegenerative diseases that are histopathologically characterized by swollen axons (spheroids). We previously established a novel rat model of neuroaxonal dystrophy harboring a V95E missense mutation in the\n                    <jats:italic toggle=\"yes\">heat shock protein family A (HSP70) member 8<\/jats:italic>\n                    (\n                    <jats:italic toggle=\"yes\">Hspa8<\/jats:italic>\n                    ) gene that originally emerged by ENU-induced random mutagenesis.\n                    <jats:italic toggle=\"yes\">Hspa8<\/jats:italic>\n                    <jats:sup>\n                      <jats:italic toggle=\"yes\">V95E<\/jats:italic>\n                    <\/jats:sup>\n                    mutant rats show marked spheroid formation in the central nervous system and develop progressive hind limb ataxia. However, the detailed pathology of\n                    <jats:italic toggle=\"yes\">Hspa8<\/jats:italic>\n                    <jats:sup>\n                      <jats:italic toggle=\"yes\">V95E<\/jats:italic>\n                    <\/jats:sup>\n                    mutant rats remains to be elucidated. In the current study, we examined the characteristics of gait abnormalities and the detailed distribution of spheroids in the central nervous system of\n                    <jats:italic toggle=\"yes\">Hspa8<\/jats:italic>\n                    <jats:sup>\n                      <jats:italic toggle=\"yes\">V95E<\/jats:italic>\n                    <\/jats:sup>\n                    knock-in (KI) rats. Using footprint tests, we demonstrated the increased ratio of step width to step length (outward step) of the hind limbs in\n                    <jats:italic toggle=\"yes\">Hspa8<\/jats:italic>\n                    <jats:sup>\n                      <jats:italic toggle=\"yes\">V95E<\/jats:italic>\n                    <\/jats:sup>\n                    KI rats from 3 weeks of age. In\n                    <jats:italic toggle=\"yes\">Hspa8<\/jats:italic>\n                    <jats:sup>\n                      <jats:italic toggle=\"yes\">V95E<\/jats:italic>\n                    <\/jats:sup>\n                    KI rats aged 15 weeks, spheroids were predominantly distributed in the proprioceptive sensory pathway from the lower body, including the gracile fasciculus and the nucleus. The number of spheroids increased with age and was accompanied by glial reactions in the dorsal funiculus. Moreover, we observed the accumulation of kinesin light chain 1; kinesin heavy chain isoforms 5A, -5B, and -5C; and microtubule-associated protein light chain 3 B in the spheroids and degenerated axons of\n                    <jats:italic toggle=\"yes\">Hspa8<\/jats:italic>\n                    <jats:sup>\n                      <jats:italic toggle=\"yes\">V95E<\/jats:italic>\n                    <\/jats:sup>\n                    KI rats at 13 weeks of age. Collectively, our data suggest the involvement of impaired axonal transport and autophagy in spheroid formation in\n                    <jats:italic toggle=\"yes\">Hspa8<\/jats:italic>\n                    <jats:sup>\n                      <jats:italic toggle=\"yes\">V95E<\/jats:italic>\n                    <\/jats:sup>\n                    mutant rats.\n                  <\/jats:p>","DOI":"10.1177\/03009858261428505","type":"journal-article","created":{"date-parts":[[2026,3,11]],"date-time":"2026-03-11T18:31:12Z","timestamp":1773253872000},"page":"927-937","update-policy":"https:\/\/doi.org\/10.1177\/sage-journals-update-policy","source":"Crossref","is-referenced-by-count":0,"title":["Characterization of the progressive neuroaxonal dystrophy and subsequent gait abnormalities in the\n                    <i>Hspa8<\/i>\n                    <sup>\n                      <i>V95E<\/i>\n                    <\/sup>\n                    knock-in rats"],"prefix":"10.1177","volume":"63","author":[{"ORCID":"https:\/\/orcid.org\/0009-0002-3302-3826","authenticated-orcid":false,"given":"Takahiro","family":"Sekiguchi","sequence":"first","affiliation":[{"name":"Osaka Metropolitan University, Izumisano, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0009-0006-8237-260X","authenticated-orcid":false,"given":"Miyuu","family":"Tanaka","sequence":"additional","affiliation":[{"name":"Osaka Metropolitan University, Izumisano, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ryoko","family":"Fujikawa","sequence":"additional","affiliation":[{"name":"Osaka Metropolitan University, Izumisano, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Takashi","family":"Kuramoto","sequence":"additional","affiliation":[{"name":"Tokyo University of Agriculture, Atsugi, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-3236-6050","authenticated-orcid":false,"given":"Takeshi","family":"Izawa","sequence":"additional","affiliation":[{"name":"Osaka Metropolitan University, Izumisano, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0009-0004-6577-2252","authenticated-orcid":false,"given":"Mitsuru","family":"Kuwamura","sequence":"additional","affiliation":[{"name":"Osaka Metropolitan University, Izumisano, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"179","published-online":{"date-parts":[[2026,3,10]]},"reference":[{"key":"e_1_3_3_2_2","doi-asserted-by":"publisher","DOI":"10.2460\/javma.239.6.823"},{"key":"e_1_3_3_3_2","doi-asserted-by":"publisher","DOI":"10.1007\/s00234-016-1726-6"},{"key":"e_1_3_3_4_2","doi-asserted-by":"publisher","DOI":"10.1186\/s13023-020-01355-2"},{"key":"e_1_3_3_5_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.jcpa.2017.02.006"},{"key":"e_1_3_3_6_2","doi-asserted-by":"publisher","DOI":"10.1172\/JCI168554"},{"key":"e_1_3_3_7_2","doi-asserted-by":"publisher","DOI":"10.1016\/0006-8993(91)91135-N"},{"key":"e_1_3_3_8_2","doi-asserted-by":"publisher","DOI":"10.1016\/S0140-6736(94)91712-4"},{"key":"e_1_3_3_9_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.jcpa.2005.10.002"},{"key":"e_1_3_3_10_2","doi-asserted-by":"publisher","DOI":"10.1177\/104063879300500414"},{"key":"e_1_3_3_11_2","unstructured":"Chopra S Tadi P. 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