{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,5,2]],"date-time":"2026-05-02T01:52:05Z","timestamp":1777686725060,"version":"3.51.4"},"reference-count":24,"publisher":"SAGE Publications","issue":"4","license":[{"start":{"date-parts":[[2021,8,18]],"date-time":"2021-08-18T00:00:00Z","timestamp":1629244800000},"content-version":"unspecified","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by-nc\/4.0\/"}],"content-domain":{"domain":["journals.sagepub.com"],"crossmark-restriction":true},"short-container-title":["Journal of Huntington's Disease"],"published-print":{"date-parts":[[2021,9,7]]},"abstract":"<jats:sec>\n                    <jats:title>Background:<\/jats:title>\n                    <jats:p>The pathological mechanism of cellular dysfunction and death in Huntington\u2019s disease (HD) is not well defined. Our transgenic HD sheep model (OVT73) was generated to investigate these mechanisms and for therapeutic testing. One particular cohort of animals has undergone focused investigation resulting in a large interrelated multi-omic dataset, with statistically significant changes observed comparing OVT73 and control \u2018omic\u2019 profiles and reported in literature.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Objective:<\/jats:title>\n                    <jats:p>Here we make this dataset publicly available for the advancement of HD pathogenic mechanism discovery.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods:<\/jats:title>\n                    <jats:p>To enable investigation in a user-friendly format, we integrated seven multi-omic datasets from a cohort of 5-year-old OVT73 ( n\u200a=\u200a6) and control ( n\u200a=\u200a6) sheep into a single database utilising the programming language R. It includes high-throughput transcriptomic, metabolomic and proteomic data from blood, brain, and other tissues.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results:<\/jats:title>\n                    <jats:p>We present the \u2018multi-omic\u2019 HD sheep database as a queryable web-based platform that can be used by the wider HD research community ( https:\/\/hdsheep.cer.auckland.ac.nz\/ ). The database is supported with a suite of simple automated statistical analysis functions for rapid exploratory analyses. We present examples of its use that validates the integrity relative to results previously reported. The data may also be downloaded for user determined analysis.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusion:<\/jats:title>\n                    <jats:p>We propose the use of this online database as a hypothesis generator and method to confirm\/refute findings made from patient samples and alternate model systems, to expand our understanding of HD pathogenesis. Importantly, additional tissue samples are available for further investigation of this cohort.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.3233\/jhd-210482","type":"journal-article","created":{"date-parts":[[2021,8,20]],"date-time":"2021-08-20T12:40:56Z","timestamp":1629463256000},"page":"423-434","update-policy":"https:\/\/doi.org\/10.1177\/sage-journals-update-policy","source":"Crossref","is-referenced-by-count":10,"title":["A Multi-Omic Huntington\u2019s Disease Transgenic Sheep-Model Database for Investigating Disease Pathogenesis"],"prefix":"10.1177","volume":"10","author":[{"given":"Emily R.","family":"Mears","sequence":"first","affiliation":[{"name":"Centre for Brain Research, School of Biological Sciences, The University of Auckland, Auckland, New Zealand"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Renee R.","family":"Handley","sequence":"additional","affiliation":[{"name":"Centre for Brain Research, School of Biological Sciences, The University of Auckland, Auckland, New Zealand"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Matthew J.","family":"Grant","sequence":"additional","affiliation":[{"name":"Centre for Brain Research, School of Biological Sciences, The University of Auckland, Auckland, New Zealand"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Suzanne J.","family":"Reid","sequence":"additional","affiliation":[{"name":"Centre for Brain Research, School of Biological Sciences, The University of Auckland, Auckland, New Zealand"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Benjamin T.","family":"Day","sequence":"additional","affiliation":[{"name":"High Performance Sport New Zealand, Mairangi Bay, Auckland, New Zealand"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Skye R.","family":"Rudiger","sequence":"additional","affiliation":[{"name":"Molecular Biology and Reproductive Technology Laboratories, South Australian Research and Development Institute, Livestock Sciences Division, Rosedale, SA, Australia"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Clive J.","family":"McLaughlan","sequence":"additional","affiliation":[{"name":"Molecular Biology and Reproductive Technology Laboratories, South Australian Research and Development Institute, Livestock Sciences Division, Rosedale, SA, Australia"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Paul J.","family":"Verma","sequence":"additional","affiliation":[{"name":"Molecular Biology and Reproductive Technology Laboratories, South Australian Research and Development Institute, Livestock Sciences Division, Rosedale, SA, Australia"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Simon C.","family":"Bawden","sequence":"additional","affiliation":[{"name":"Molecular Biology and Reproductive Technology Laboratories, South Australian Research and Development Institute, Livestock Sciences Division, Rosedale, SA, Australia"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Stefano","family":"Patassini","sequence":"additional","affiliation":[{"name":"Centre for Brain Research, School of Biological Sciences, The University of Auckland, Auckland, New Zealand"},{"name":"Centre for Advanced Discovery and Experimental Therapeutics (CADET), Central Manchester University Hospitals NHS Foundation Trust, Manchester Academic Health Sciences Centre, Manchester, UK"},{"name":"Division of Cardiovascular Science, Faculty of Biology, Medicine and Health, The University of Manchester, Manchester, UK"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Richard D.","family":"Unwin","sequence":"additional","affiliation":[{"name":"Centre for Advanced Discovery and Experimental Therapeutics (CADET), Central Manchester University Hospitals NHS Foundation Trust, Manchester Academic Health Sciences Centre, Manchester, UK"},{"name":"Division of Cardiovascular Science, Faculty of Biology, Medicine and Health, The University of Manchester, Manchester, UK"},{"name":"Stoller Biomarker Discovery Centre, Division of Cancer Sciences, School of Medical Sciences, Faculty of Biology, Medicine and Health, The University of Manchester, Manchester, UK"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Garth J.S.","family":"Cooper","sequence":"additional","affiliation":[{"name":"Centre for Brain Research, School of Biological Sciences, The University of Auckland, Auckland, New Zealand"},{"name":"Centre for Advanced Discovery and Experimental Therapeutics (CADET), Central Manchester University Hospitals NHS Foundation Trust, Manchester Academic Health Sciences Centre, Manchester, UK"},{"name":"Division of Cardiovascular Science, Faculty of Biology, Medicine and Health, The University of Manchester, Manchester, UK"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"James F.","family":"Gusella","sequence":"additional","affiliation":[{"name":"Molecular Neurogenetics Unit, Center for Genomic Medicine, Massachusetts General Hospital, Boston, MA, USA"},{"name":"Department of Genetics, Harvard Medical School, Boston, MA, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Marcy E.","family":"MacDonald","sequence":"additional","affiliation":[{"name":"Molecular Neurogenetics Unit, Center for Genomic Medicine, Massachusetts General Hospital, Boston, MA, USA"},{"name":"Department of Neurology, Harvard Medical School, Boston, MA, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Rudiger","family":"Brauning","sequence":"additional","affiliation":[{"name":"Invermay Agricultural Centre, AgResearch Ltd., Mosgiel, New Zealand"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Paul","family":"Maclean","sequence":"additional","affiliation":[{"name":"Invermay Agricultural Centre, AgResearch Ltd., Mosgiel, New Zealand"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"John F.","family":"Pearson","sequence":"additional","affiliation":[{"name":"Biostatistics and Computational Biology Unit, University of Otago, Christchurch, New Zealand"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Henry J.","family":"Waldvogel","sequence":"additional","affiliation":[{"name":"Centre for Brain Research, Faculty of Medical and Health Science, The University of Auckland, Auckland, New Zealand"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Richard L.M.","family":"Faull","sequence":"additional","affiliation":[{"name":"Centre for Brain Research, Faculty of Medical and Health Science, The University of Auckland, Auckland, New Zealand"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Russell G.","family":"Snell","sequence":"additional","affiliation":[{"name":"Centre for Brain Research, School of Biological Sciences, The University of Auckland, Auckland, New Zealand"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"179","published-online":{"date-parts":[[2021,8,18]]},"reference":[{"key":"ref001","doi-asserted-by":"publisher","DOI":"10.1016\/0092-8674(93)90585-E"},{"key":"ref002","doi-asserted-by":"publisher","DOI":"10.1038\/ng0893-398"},{"key":"ref003","doi-asserted-by":"publisher","DOI":"10.1038\/ng0893-387"},{"key":"ref004","doi-asserted-by":"publisher","DOI":"10.1038\/ng0893-393"},{"key":"ref005","doi-asserted-by":"publisher","DOI":"10.1016\/s0165-6147(00)01884-8"},{"key":"ref006","doi-asserted-by":"publisher","DOI":"10.1093\/hmg\/ddq063"},{"key":"ref007","doi-asserted-by":"publisher","DOI":"10.3233\/JHD-130067"},{"key":"ref008","doi-asserted-by":"publisher","DOI":"10.1093\/hmg\/ddu047"},{"key":"ref009","doi-asserted-by":"publisher","DOI":"10.1016\/j.expneurol.2012.10.014"},{"key":"ref010","doi-asserted-by":"publisher","DOI":"10.1038\/srep20681"},{"key":"ref011","doi-asserted-by":"publisher","DOI":"10.1038\/srep43030"},{"key":"ref012","doi-asserted-by":"publisher","DOI":"10.1093\/brain\/awl027"},{"key":"ref013","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.1711243115"},{"key":"ref014","doi-asserted-by":"publisher","DOI":"10.1093\/hmg\/ddl013"},{"key":"ref015","doi-asserted-by":"publisher","DOI":"10.1016\/j.bbrc.2015.10.140"},{"key":"ref016","doi-asserted-by":"publisher","DOI":"10.1038\/ng.2202"},{"key":"ref017","unstructured":"PatassiniS. Discovery and validation of relevant markers of Huntington\u2019s disease progression using a transgenic sheep model. Auckland, New Zealand: The University of Auckland (PhD Thesis); 2014."},{"key":"ref018","doi-asserted-by":"publisher","DOI":"10.21105\/joss.01686"},{"key":"ref019","doi-asserted-by":"publisher","DOI":"10.18637\/jss.v059.i10"},{"key":"ref020","unstructured":"RStudio Team. RStudio: Integrated Development Environment for R. Boston, MA: RStudio, Inc.; 2018. Available from: http:\/\/www.rstudio.com\/"},{"key":"ref021","doi-asserted-by":"publisher","DOI":"10.1038\/nn.4256"},{"key":"ref022","unstructured":"Enroll-HD. Enroll-HD: A user guide to the clinical datasets and biosamples available from Enroll-HD. CHDI Foundation. 2016;Version 2016-10-R1."},{"key":"ref023","doi-asserted-by":"publisher","DOI":"10.1038\/s41598-017-05224-0"},{"key":"ref024","doi-asserted-by":"publisher","DOI":"10.1038\/s42003-018-0254-9"}],"container-title":["Journal of Huntington's Disease"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/journals.sagepub.com\/doi\/pdf\/10.3233\/JHD-210482","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/journals.sagepub.com\/doi\/full-xml\/10.3233\/JHD-210482","content-type":"application\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/journals.sagepub.com\/doi\/pdf\/10.3233\/JHD-210482","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,4,29]],"date-time":"2026-04-29T08:56:02Z","timestamp":1777452962000},"score":1,"resource":{"primary":{"URL":"https:\/\/journals.sagepub.com\/doi\/10.3233\/JHD-210482"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2021,8,18]]},"references-count":24,"journal-issue":{"issue":"4","published-print":{"date-parts":[[2021,9,7]]}},"alternative-id":["10.3233\/JHD-210482"],"URL":"https:\/\/doi.org\/10.3233\/jhd-210482","relation":{},"ISSN":["1879-6397","1879-6400"],"issn-type":[{"value":"1879-6397","type":"print"},{"value":"1879-6400","type":"electronic"}],"subject":[],"published":{"date-parts":[[2021,8,18]]}}}