{"id":20127,"date":"2024-03-31T10:25:03","date_gmt":"2024-03-31T14:25:03","guid":{"rendered":"https:\/\/arsacs.com\/?p=20127"},"modified":"2026-03-16T18:16:06","modified_gmt":"2026-03-16T22:16:06","slug":"physiological-and-behavioral-effects-of-omaveloxolone-in-an-arsacs-mouse-model-drs-schmahmann-and-lin","status":"publish","type":"post","link":"https:\/\/2026.arsacs.com\/fr\/physiological-and-behavioral-effects-of-omaveloxolone-in-an-arsacs-mouse-model-drs-schmahmann-and-lin\/","title":{"rendered":"\u201cPhysiological and behavioral effects of Omaveloxolone in an ARSACS mouse model\u201d- Drs. Schmahmann et Lin"},"content":{"rendered":"<p>Autosomal-recessive spastic ataxia of Charlevoix-Saguenay (ARSACS) is a relentlessly progressing genetic ataxia with no known treatment. A major mechanism underlies the pathogenesis of ARSACS is mitochondrial dysfunction that causes oxidative stress, resulting in neurodegeneration. Incidentally, mitochondrial dysfunction is also a pathogenic mechanism in another genetic ataxia, Friedreich\u2019s ataxia (FA). Recent studies and clinical trials have demonstrated that omaveloxolone (brand name Skyclarys) can improve clinical symptoms in FA via enhancing the ability of mitochondria to cope with oxidative stress. This has led to approval by the FDA of Skyclarys for the treatment of FA.<\/p>\n\n\n\n<p>Our team plans to administer Skyclarys to a mouse model of ARSACS and investigate whether Skyclarys can change the disease course in these mice. We believe the findings of our proposed study will be critical to determining whether omaveloxolone is appropriate for clinical trials in ARSACS.<\/p>\n\n\n\n<p><strong>Ce projet est financ\u00e9 conjointement par la Fondation de l'Ataxie Charlevoix- Saguenay  et le  Massachusetts General ARSACS Fundraiser<\/strong>, <strong>fonds cr\u00e9\u00e9 par des familles aux E. U. <\/strong><\/p>\n\n\n\n<p>Financement : 100 000$ USD<\/p>\n\n\n\n<p>Dur\u00e9e: 1an <\/p>\n\n\n\n<p>Coordonn\u00e9es:<\/p>\n\n\n\n<p><\/p>\n\n\n\n<p><\/p>\n\n\n\n<figure class=\"wp-block-image size-full is-resized\"><img loading=\"lazy\" decoding=\"async\" width=\"460\" height=\"460\" src=\"https:\/\/2026.arsacs.com\/wp-content\/uploads\/2024\/03\/Jeremy_Schmahmann.jpg\" alt=\"\" class=\"wp-image-20072\" style=\"width:164px;height:auto\" srcset=\"https:\/\/2026.arsacs.com\/wp-content\/uploads\/2024\/03\/Jeremy_Schmahmann.jpg 460w, https:\/\/2026.arsacs.com\/wp-content\/uploads\/2024\/03\/Jeremy_Schmahmann-300x300.jpg 300w, https:\/\/2026.arsacs.com\/wp-content\/uploads\/2024\/03\/Jeremy_Schmahmann-150x150.jpg 150w\" sizes=\"(max-width: 460px) 100vw, 460px\" \/><\/figure>\n\n\n\n<p class=\"has-text-align-left\">Dr. Jeremy Schmahmann,&nbsp; M.D. <\/p>\n\n\n\n<p class=\"has-text-align-left\">Professor of Neurology<\/p>\n\n\n\n<p class=\"has-text-align-left\">Harvard Medical School<\/p>\n\n\n\n<p class=\"has-text-align-left\">Massachusetts General Hospital&nbsp;<\/p>\n\n\n\n<p class=\"has-text-align-left\">100 Cambridge Street, Suite 2000, Boston, MA 02114<br>jschmahmann@mgh.harvard.edu       <\/p>\n\n\n\n<p class=\"has-text-align-center\"><\/p>\n\n\n\n<figure class=\"wp-block-image size-full is-resized\"><img loading=\"lazy\" decoding=\"async\" width=\"601\" height=\"600\" src=\"https:\/\/2026.arsacs.com\/wp-content\/uploads\/2024\/03\/Lin_Chih-Chun_DSC04079-cropped-2x22-1.jpg\" alt=\"\" class=\"wp-image-20076\" style=\"width:183px;height:auto\" srcset=\"https:\/\/2026.arsacs.com\/wp-content\/uploads\/2024\/03\/Lin_Chih-Chun_DSC04079-cropped-2x22-1.jpg 601w, https:\/\/2026.arsacs.com\/wp-content\/uploads\/2024\/03\/Lin_Chih-Chun_DSC04079-cropped-2x22-1-480x479.jpg 480w\" sizes=\"(min-width: 0px) and (max-width: 480px) 480px, (min-width: 481px) 601px, 100vw\" \/><\/figure>\n\n\n\n<p class=\"has-text-align-left\">Dr Chih-Chun Lin,&nbsp; M.D.<\/p>\n\n\n\n<p class=\"has-text-align-left\">Massachusetts General Hospital&nbsp;<\/p>\n\n\n\n<p class=\"has-text-align-left\">100 Cambridge Street, Suite 2000<br>Boston, MA 02114<br>clin69@mgh.harvard.edu<\/p>\n\n\n\n<p class=\"has-text-align-left\"><\/p>\n\n\n\n<p class=\"has-text-align-center\">     <\/p>\n\n\n\n<p class=\"has-text-align-left\"><\/p>\n\n\n\n<p><\/p>\n\n\n\n<p class=\"has-text-align-center\"> <\/p>\n\n\n\n<p><\/p>\n\n\n\n<p><\/p>\n\n\n\n<p class=\"has-text-align-center\"><\/p>\n\n\n\n<p class=\"has-text-align-center\"><\/p>\n\n\n\n<p class=\"has-text-align-center\"><\/p>\n\n\n\n<p class=\"has-text-align-left\"><\/p>\n\n\n\n<p><\/p>\n\n\n\n<p>Dr. Chih-Chun Lin, M.D.<\/p>\n\n\n\n<p>Harvard Medical School<\/p>\n\n\n\n<p>Massachusetts General Hospital&nbsp;<\/p>\n\n\n\n<p>100 Cambridge Street, Suite 2000<br>Boston, MA 02114<br>clin69@mgh.harvard.edu<\/p>\n\n\n\n<p><\/p>","protected":false},"excerpt":{"rendered":"<p>Autosomal-recessive spastic ataxia of Charlevoix-Saguenay (ARSACS) is a relentlessly progressing genetic ataxia with no known treatment. A major mechanism underlies the pathogenesis of ARSACS is mitochondrial dysfunction that causes oxidative stress, resulting in neurodegeneration. Incidentally, mitochondrial dysfunction is also a pathogenic mechanism in another genetic ataxia, Friedreich\u2019s ataxia (FA). Recent studies and clinical trials have [&#8230;]<\/p>\n","protected":false},"author":3,"featured_media":0,"comment_status":"closed","ping_status":"closed","sticky":false,"template":"","format":"standard","meta":{"footnotes":""},"categories":[91],"tags":[],"class_list":["post-20127","post","type-post","status-publish","format-standard","hentry","category-recherche-2024"],"_links":{"self":[{"href":"https:\/\/2026.arsacs.com\/fr\/wp-json\/wp\/v2\/posts\/20127","targetHints":{"allow":["GET"]}}],"collection":[{"href":"https:\/\/2026.arsacs.com\/fr\/wp-json\/wp\/v2\/posts"}],"about":[{"href":"https:\/\/2026.arsacs.com\/fr\/wp-json\/wp\/v2\/types\/post"}],"author":[{"embeddable":true,"href":"https:\/\/2026.arsacs.com\/fr\/wp-json\/wp\/v2\/users\/3"}],"replies":[{"embeddable":true,"href":"https:\/\/2026.arsacs.com\/fr\/wp-json\/wp\/v2\/comments?post=20127"}],"version-history":[{"count":21,"href":"https:\/\/2026.arsacs.com\/fr\/wp-json\/wp\/v2\/posts\/20127\/revisions"}],"predecessor-version":[{"id":24965,"href":"https:\/\/2026.arsacs.com\/fr\/wp-json\/wp\/v2\/posts\/20127\/revisions\/24965"}],"wp:attachment":[{"href":"https:\/\/2026.arsacs.com\/fr\/wp-json\/wp\/v2\/media?parent=20127"}],"wp:term":[{"taxonomy":"category","embeddable":true,"href":"https:\/\/2026.arsacs.com\/fr\/wp-json\/wp\/v2\/categories?post=20127"},{"taxonomy":"post_tag","embeddable":true,"href":"https:\/\/2026.arsacs.com\/fr\/wp-json\/wp\/v2\/tags?post=20127"}],"curies":[{"name":"wp","href":"https:\/\/api.w.org\/{rel}","templated":true}]}}