{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,25]],"date-time":"2026-09-25T01:19:13Z","timestamp":1790299153908,"version":"4.1.0"},"reference-count":36,"publisher":"Wiley","issue":"4","license":[{"start":{"date-parts":[[2014,11,6]],"date-time":"2014-11-06T00:00:00Z","timestamp":1415232000000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"funder":[{"DOI":"10.13039\/501100001868","name":"National Science Council","doi-asserted-by":"crossref","award":["102-2627-B-006-002-"],"award-info":[{"award-number":["102-2627-B-006-002-"]}],"id":[{"id":"10.13039\/501100001868","id-type":"DOI","asserted-by":"crossref"}]},{"DOI":"10.13039\/501100001868","name":"National Science Council","doi-asserted-by":"crossref","award":["NSC 102-2628-B-006-010-MY3"],"award-info":[{"award-number":["NSC 102-2628-B-006-010-MY3"]}],"id":[{"id":"10.13039\/501100001868","id-type":"DOI","asserted-by":"crossref"}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Brain Pathology"],"published-print":{"date-parts":[[2015,7]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:p>\n                    <jats:styled-content style=\"fixed-case\">H<\/jats:styled-content>\n                    untington's disease (\n                    <jats:styled-content style=\"fixed-case\">HD<\/jats:styled-content>\n                    ) is a genetic and neurodegenerative disease, leading to motor and cognitive dysfunction in\n                    <jats:styled-content style=\"fixed-case\">HD<\/jats:styled-content>\n                    patients. At cellular level, this disease is caused by the accumulation of mutant huntingtin (\n                    <jats:styled-content style=\"fixed-case\">HTT<\/jats:styled-content>\n                    ) in different cells, and finally results in the dysfunction of different cells. To clean these mutant proteins, ubiquitin\u2010proteasome system (\n                    <jats:styled-content style=\"fixed-case\">UPS<\/jats:styled-content>\n                    ) and autophagy system are two critical pathways in the brain; however, little is known in other peripheral tissues. As mutant\n                    <jats:styled-content style=\"fixed-case\">HTT<\/jats:styled-content>\n                    affects different tissues progressively and might influence the\n                    <jats:styled-content style=\"fixed-case\">UPS<\/jats:styled-content>\n                    and autophagy pathways at early stages, we attempted to examine two clearance systems in\n                    <jats:styled-content style=\"fixed-case\">HD<\/jats:styled-content>\n                    models before the onset. Here,\n                    <jats:italic>in vitro<\/jats:italic>\n                    results showed that the accumulation of\n                    <jats:styled-content style=\"fixed-case\">UPS<\/jats:styled-content>\n                    signals with time was observed obviously in neuroblastoma and kidney cells, not in other cells. In\n                    <jats:styled-content style=\"fixed-case\">HD<\/jats:styled-content>\n                    transgenic mice, we observed the impairment of\n                    <jats:styled-content style=\"fixed-case\">UPS<\/jats:styled-content>\n                    , but not autophagy, over time in the cortex and striatum. In heart and muscle tissues, disturbance of autophagy was observed, whereas dysfunction of\n                    <jats:styled-content style=\"fixed-case\">UPS<\/jats:styled-content>\n                    was displayed in liver and lung. These results suggest that two protein clearance pathways are disturbed differentially in different tissues before the onset of\n                    <jats:styled-content style=\"fixed-case\">HD<\/jats:styled-content>\n                    , and enhancement of protein clearance at early stages might provide a potential stratagem to alleviate the progression of\n                    <jats:styled-content style=\"fixed-case\">HD<\/jats:styled-content>\n                    .\n                  <\/jats:p>","DOI":"10.1111\/bpa.12191","type":"journal-article","created":{"date-parts":[[2014,9,1]],"date-time":"2014-09-01T23:09:13Z","timestamp":1409612953000},"page":"481-490","source":"Crossref","is-referenced-by-count":13,"title":["The Differential Profiling of Ubiquitin\u2010Proteasome and Autophagy Systems in Different Tissues before the Onset of\n                    <scp>H<\/scp>\n                    untington's Disease Models"],"prefix":"10.1111","volume":"25","author":[{"given":"Lu\u2010Shiun","family":"Her","sequence":"first","affiliation":[{"name":"Department of Life Sciences College of Bioscience and Biotechnology National Cheng Kung University  Tainan Taiwan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jian\u2010Yu","family":"Lin","sequence":"additional","affiliation":[{"name":"Department of Physiology National Cheng Kung University  Tainan Taiwan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mu\u2010Hui","family":"Fu","sequence":"additional","affiliation":[{"name":"Institute of Basic Medical Sciences College of Medicine National Cheng Kung University  Tainan Taiwan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yu\u2010Fan","family":"Chang","sequence":"additional","affiliation":[{"name":"Department of Physiology National Cheng Kung University  Tainan 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