{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,12,16]],"date-time":"2025-12-16T12:49:56Z","timestamp":1765889396503,"version":"3.37.3"},"reference-count":73,"publisher":"Oxford University Press (OUP)","issue":"24","license":[{"start":{"date-parts":[[2024,10,30]],"date-time":"2024-10-30T00:00:00Z","timestamp":1730246400000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"DOI":"10.13039\/100000002","name":"US National Institutes of Health","doi-asserted-by":"publisher","award":["R01 NS085054"],"award-info":[{"award-number":["R01 NS085054"]}],"id":[{"id":"10.13039\/100000002","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100000065","name":"National Institute of Neurological Disorders and Stroke","doi-asserted-by":"publisher","award":["R01 NS128285","R37 NS022920"],"award-info":[{"award-number":["R01 NS128285","R37 NS022920"]}],"id":[{"id":"10.13039\/100000065","id-type":"DOI","asserted-by":"publisher"}]},{"name":"National Ataxia Foundation Research Seed Money Grant Award"},{"name":"National Ataxia Foundation Pioneer Award"},{"name":"Wallin Neuroscience Discovery Award"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2024,12,6]]},"abstract":"<jats:title>Abstract<\/jats:title>\n               <jats:p>One of the characteristic regions of brainstem degeneration across multiple spinocerebellar ataxias (SCAs) is the inferior olive (IO), a medullary nucleus that plays a key role in motor learning. The vulnerability of IO neurons remains a poorly-understood area of SCA pathology. In this work, we address this by evaluating IO disease in SCA1, a prototypic inherited olivopontocerebellar atrophy, using the genetically-precise SCA1 knock-in (SCA1-KI) mouse. We find that these mice exhibit olivary hypertrophy, a phenotype reminiscent of a degenerative disorder known as hypertrophic olivary degeneration (HOD). Similar to early stages of HOD, SCA1-KI IO neurons display early dendritic lengthening and later somatic expansion without frank cell loss. Though HOD is known to be caused by brainstem lesions that disrupt IO inhibitory innervation, we observe no loss of inhibitory terminals in the SCA1-KI IO. Additionally, we find that a separate mouse model of SCA1 in which mutant ATXN1 is expressed solely in cerebellar Purkinje cells shows no evidence of olivary hypertrophy. Patch-clamp recordings from brainstem slices indicate that SCA1-KI IO neurons are hyperexcitable, generating spike trains in response to membrane depolarization. Transcriptome analysis further reveals reduced medullary expression of ion channels responsible for IO neuron spike afterhyperpolarization (AHP)\u2014a result that appears to have a functional consequence, as SCA1-KI IO neuron spikes exhibit a diminished AHP. These findings suggest that expression of mutant ATXN1 in IO neurons results in an HOD-like olivary hypertrophy, in association with increased intrinsic membrane excitability and ion channel transcriptional dysregulation.<\/jats:p>","DOI":"10.1093\/hmg\/ddae146","type":"journal-article","created":{"date-parts":[[2024,10,30]],"date-time":"2024-10-30T16:17:00Z","timestamp":1730305020000},"page":"2159-2176","source":"Crossref","is-referenced-by-count":1,"title":["Increased intrinsic membrane excitability is associated with olivary hypertrophy in spinocerebellar ataxia type 1"],"prefix":"10.1093","volume":"33","author":[{"given":"Logan M","family":"Morrison","sequence":"first","affiliation":[{"name":"Neuroscience Graduate Program, University of Michigan , Ann Arbor, MI 48109,","place":["United States"]},{"name":"Peter O\u2019Donnell Jr. Brain Institute, University of Texas Southwestern Medical Center , 6124 Harry Hines Blvd. Dallas, TX 75390,","place":["United States"]},{"name":"Department of Neurology, University of Texas Southwestern Medical Center , Dallas, 5323 Harry Hines Blvd., TX 75390,","place":["United States"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Haoran","family":"Huang","sequence":"additional","affiliation":[{"name":"Medical Scientist Training Program, The Ohio State University , Columbus, OH 43210,","place":["United States"]},{"name":"College of Medicine, The Ohio State University , Columbus, OH 43210,","place":["United States"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Hillary P","family":"Handler","sequence":"additional","affiliation":[{"name":"Molecular Diagnostics Laboratory, University of Minnesota Fairview Medical Center , Minneapolis, MN 55455,","place":["United States"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Min","family":"Fu","sequence":"additional","affiliation":[{"name":"Peter O\u2019Donnell Jr. Brain Institute, University of Texas Southwestern Medical Center , 6124 Harry Hines Blvd. Dallas, TX 75390,","place":["United States"]},{"name":"Department of Neurology, University of Texas Southwestern Medical Center , Dallas, 5323 Harry Hines Blvd., TX 75390,","place":["United States"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Deborah M","family":"Jones","sequence":"additional","affiliation":[{"name":"Department of Neurology, University of Texas Southwestern Medical Center , Dallas, 5323 Harry Hines Blvd., TX 75390,","place":["United States"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"David D","family":"Bushart","sequence":"additional","affiliation":[{"name":"College of Medicine, The Ohio State University , Columbus, OH 43210,","place":["United States"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-6980-2058","authenticated-orcid":false,"given":"Samuel S","family":"Pappas","sequence":"additional","affiliation":[{"name":"Peter O\u2019Donnell Jr. Brain Institute, University of Texas Southwestern Medical Center , 6124 Harry Hines Blvd. Dallas, TX 75390,","place":["United States"]},{"name":"Department of Neurology, University of Texas Southwestern Medical Center , Dallas, 5323 Harry Hines Blvd., TX 75390,","place":["United States"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Harry T","family":"Orr","sequence":"additional","affiliation":[{"name":"Institute for Translational Neuroscience, University of Minnesota , Minneapolis, 420 Delaware Street SE, MN 55455,","place":["United States"]},{"name":"Department of Laboratory Medicine and Pathology, University of Minnesota , 420 Delaware Street SE, Minneapolis, MN 55455,","place":["United States"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-2742-6469","authenticated-orcid":false,"given":"Vikram G","family":"Shakkottai","sequence":"additional","affiliation":[{"name":"Peter O\u2019Donnell Jr. Brain Institute, University of Texas Southwestern Medical Center , 6124 Harry Hines Blvd. Dallas, TX 75390,","place":["United States"]},{"name":"Department of Neurology, University of Texas Southwestern Medical Center , Dallas, 5323 Harry Hines Blvd., TX 75390,","place":["United States"]}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"286","published-online":{"date-parts":[[2024,10,30]]},"reference":[{"key":"2024121021340609200_ref1","doi-asserted-by":"crossref","first-page":"230","DOI":"10.1007\/s12311-016-0787-8","article-title":"The roles of the Olivocerebellar pathway in motor learning and motor control","volume":"16","author":"Lang","year":"2017","journal-title":"Cerebellum"},{"key":"2024121021340609200_ref2","doi-asserted-by":"crossref","first-page":"391","DOI":"10.1016\/S0166-2236(98)01310-1","article-title":"Microcircuitry and function of the inferior olive","volume":"21","author":"De Zeeuw","year":"1998","journal-title":"Trends 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