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Psychosine is believed to cause central demyelination by killing oligodendrocytes. Quantitative analysis of this process is lacking. To address this, we generated a new transgenic reporter twitcher line in which myelinating oligodendrocytes are genetically marked by the expression of LacZ under control of the myelin basic protein (MBP) promoter. MBP\u2010LacZ\u2010twitcher transgenic mice were used for unbiased stereological quantification of \u03b2\u2010galactosidase<jats:sup>+<\/jats:sup> oligodendrocytes in the spinal cord. As expected, we found decreased numbers of these cells in mutant cords, paralleling the severity of clinical disease. The decrease of oligodendrocytes does not correlate well with the increase of psychosine. The new MBP\u2010LacZ\u2010twitcher line will be a useful genetic tool for measuring changes in oligodendrocyte numbers in different regions of the mutant CNS and in preclinical trials of therapies to prevent demyelination. \u00a9 2016 Wiley Periodicals, Inc.<\/jats:p>","DOI":"10.1002\/jnr.23839","type":"journal-article","created":{"date-parts":[[2016,7,18]],"date-time":"2016-07-18T05:16:43Z","timestamp":1468819003000},"page":"1520-1530","source":"Crossref","is-referenced-by-count":2,"title":["Generation of a LacZ reporter transgenic mouse line for the stereological analysis of oligodendrocyte loss in galactosylceramidase deficiency"],"prefix":"10.1002","volume":"94","author":[{"given":"Hongling","family":"Zhu","sequence":"first","affiliation":[{"name":"Department of Anatomy and Cell Biology College of Medicine University of Illinois at Chicago Chicago Illinois"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Francesca","family":"Ornaghi","sequence":"additional","affiliation":[{"name":"San Raffaele Scientific Institute Milano Italy"},{"name":"Hunter James Kelly Research Institute University at Buffalo School of Medicine and Biomedical Sciences Buffalo New York"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Sophie","family":"Belin","sequence":"additional","affiliation":[{"name":"Hunter James Kelly Research Institute University at Buffalo School of Medicine and Biomedical Sciences Buffalo New York"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Maria I.","family":"Givogri","sequence":"additional","affiliation":[{"name":"Department of Anatomy and Cell Biology College of Medicine University of Illinois at Chicago Chicago Illinois"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Lawrence","family":"Wrabetz","sequence":"additional","affiliation":[{"name":"Hunter James Kelly Research Institute University at Buffalo School of Medicine and Biomedical Sciences Buffalo New York"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ernesto R.","family":"Bongarzone","sequence":"additional","affiliation":[{"name":"Department of Anatomy and Cell Biology College of Medicine University of Illinois at Chicago Chicago Illinois"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2016,7,18]]},"reference":[{"key":"e_1_2_7_2_1","first-page":"861","article-title":"Peripheral neuropathy in globoid cell leukodystrophy (Krabbe's disease)","volume":"92","author":"Bischoff A","year":"1969","journal-title":"Ultrastructural and histochemical findings. 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