{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,8,2]],"date-time":"2025-08-02T04:46:58Z","timestamp":1754110018716,"version":"3.37.3"},"reference-count":69,"publisher":"Wiley","issue":"1","license":[{"start":{"date-parts":[[2014,9,19]],"date-time":"2014-09-19T00:00:00Z","timestamp":1411084800000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"funder":[{"DOI":"10.13039\/100000002","name":"National Institutes of Health","doi-asserted-by":"publisher","award":["NS064195"],"award-info":[{"award-number":["NS064195"]}],"id":[{"id":"10.13039\/100000002","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["J of Neuroscience Research"],"published-print":{"date-parts":[[2015,1]]},"abstract":"<jats:p>Low vitamin D level is a risk factor for various late\u2010onset CNS demyelinating disorders. We investigated whether vitamin D deficiency influences disease in <jats:italic>twitcher<\/jats:italic> mice (<jats:italic>GALC<\/jats:italic><jats:sup>twi\/twi<\/jats:sup>; <jats:italic>twi<\/jats:italic>), a murine model of Krabbe disease (KD), an inherited disorder caused by galactocerebrosidase (GALC) deficiency that leads to psychosine accumulation, oligodendrocyte (OL) loss, and CNS demyelination. We found that the in situ 1,25\u2010dihydroxyvitamin D3 level was reduced, with a parallel increase in the expression of inflammatory cytokines and vitamin D\u2010catabolizing enzymes in the brains of KD and <jats:italic>twi<\/jats:italic> mice compared with age\u2010matched controls. Pups maintained on milk from lactating heterozygous (<jats:italic>GALC<\/jats:italic><jats:sup>twi\/+<\/jats:sup>) mothers that were fed a vitamin D3\u2010supplemented diet until weaning and then fed a vitamin D3\u2010supplemented diet demonstrated delayed body weight loss and development of disease in <jats:italic>twi<\/jats:italic> mice. This delayed the onset of tremors and locomotor disabilities that eventually impacted the life span of <jats:italic>twi<\/jats:italic> mice (50\u2009\u00b1\u20092 days). Accordingly, the expression of antioxidant enzymes was increased with delayed psychosine accumulation, lipid peroxidation, and inflammatory response that eventually protected CNS myelin and axonal integrity in <jats:italic>twi<\/jats:italic> mice. In vitro studies revealed that 1,25\u2010dihydroxyvitamin D3 enhances antioxidant defenses in OLs deficient for GALC or incubated with psychosine. Together these data provide the first evidence that vitamin D deficiency affects disease development in <jats:italic>twi<\/jats:italic> mice and that vitamin D3 supplementation has the potential to improve the efficacy of KD therapeutics. \u00a9 2014 Wiley Periodicals, Inc.<\/jats:p>","DOI":"10.1002\/jnr.23476","type":"journal-article","created":{"date-parts":[[2014,9,19]],"date-time":"2014-09-19T04:45:35Z","timestamp":1411101935000},"page":"28-42","source":"Crossref","is-referenced-by-count":12,"title":["Effect of vitamin D3 intake on the onset of disease in a murine model of human Krabbe disease"],"prefix":"10.1002","volume":"93","author":[{"given":"Manjeet K.","family":"Paintlia","sequence":"first","affiliation":[{"name":"Darby Children's Research Institute, Department of Pediatrics Medical University of South Carolina Charleston South Carolina"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Inderjit","family":"Singh","sequence":"additional","affiliation":[{"name":"Darby Children's Research Institute, Department of Pediatrics Medical University of South Carolina Charleston South Carolina"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Avtar K.","family":"Singh","sequence":"additional","affiliation":[{"name":"Darby Children's Research Institute, Department of Pediatrics Medical University of South Carolina Charleston South Carolina"},{"name":"Department of Pathology and Laboratory Medicine Medical University of South Carolina Charleston South Carolina"},{"name":"Ralph H. 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