{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,29]],"date-time":"2026-09-29T08:48:15Z","timestamp":1790671695300,"version":"4.1.0"},"reference-count":33,"publisher":"Wiley","issue":"3","license":[{"start":{"date-parts":[[2005,6,20]],"date-time":"2005-06-20T00:00:00Z","timestamp":1119225600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Glia"],"published-print":{"date-parts":[[2005,11,15]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>Galactosylceramide (GalC) is the major sphingolipid of the myelin membrane. Mice lacking GalC due to ceramide galactosyltransferase (CGT) deficiency form unstable and functionally affected myelin and exhibit a progressive demyelination, accompanied by severe motor coordination deficits. In addition to oligodendrocytes, CGT is also expressed in other cells, e.g., neurons and astrocytes. We examined the possibility that lack of CGT in these cells contributes to the phenotype of CGT\u2010deficient mice. Toward this aim, we generated transgenic mice expressing CGT under the control of oligodendrocyte\u2010specific proteolipid protein (PLP) promoter and examined the possibility of a transgenic rescue of CGT\u2010deficient mice. CGT\u2010deficient mice expressing the PLP\u2010CGT transgene did not show any behavioral abnormalities, normal myelin structure, and MBP levels. CGT activity as well as GalC and sulfatide levels of rescued mice were not significantly different from wild\u2010type controls. Thus, transgenic rescue with the PLP\u2010CGT transgene was apparently complete. In contrast to wild\u2010type and rescued mice, PLP\u2010CGT transgenic mice on a wild\u2010type background exhibited significantly elevated CGT activity which directly correlated with an increase in non\u2010hydroxy fatty acid (NFA)\u2010GalC, but not \u03b1\u2010hydroxy fatty acid (HFA)\u2010GalC. HFA\u2010GalC decreased in adult transgenic mice, indicating that NFA\u2010GalC, but not HFA\u2010GalC levels are limited by CGT activity. As a consequence, the total amount of GalC is unchanged over a rather wide range of CGT expression levels in the mouse brain. Our results indicate that loss of CGT in oligodendrocytes is exclusively responsible for the myelin structural deficits, demyelination, and behavioral abnormalities in CGT\u2010deficient mice. \u00a9 2005 Wiley\u2010Liss, Inc.<\/jats:p>","DOI":"10.1002\/glia.20230","type":"journal-article","created":{"date-parts":[[2005,6,20]],"date-time":"2005-06-20T19:30:40Z","timestamp":1119295840000},"page":"190-198","source":"Crossref","is-referenced-by-count":43,"title":["Oligodendrocyte\u2010specific ceramide galactosyltransferase (CGT) expression phenotypically rescues CGT\u2010deficient mice and demonstrates that CGT activity does not limit brain galactosylceramide 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