Article
Generation of a LacZ reporter transgenic mouse line for the stereological analysis of oligodendrocyte loss in galactosylceramidase deficiency.
Journal of neuroscience research - 1 Dec 2016
Zhu Hongling, Ornaghi Francesca, Belin Sophie, Givogri Maria I, Wrabetz Lawrence, Bongarzone Ernesto R
Abstract excerpt
Krabbe's disease is a leukodystrophy resulting from deficiency of galactosylceramidase and the accumulation of galactosylsphingosine (psychosine) in the nervous system. 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...
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