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Myelin deterioration in twitcher mice: Motor evoked potentials and magnetic resonance imaging as in vivo monitoring tools
Myelin deterioration in twitcher mice: Motor evoked potentials and magnetic resonance imaging as in vivo monitoring tools JOURNAL OF NEUROSCIENCE RESEARCH Dolcetta, D., Amadio, S., Guerrini, U., Givogri, M. I., Perani, L., Galbiati, F., Sironi, L., Del Carro, U., Roncarolo, M. G., Bongarzone, E. 2005; 81 (4): 597-604Abstract
We have used magnetic resonance imaging (MRI) and motor evoked potentials (MEPs) for monitoring disease progression within the CNS of the Twitcher mouse, the murine model for globoid cell leukodystrophy (GLD). GLD is a lysosomal storage disorder, resulting from galactocerebrosidase deficiency, causing central and peripheral myelin impairment, leading to death, usually during early infancy. Neuroradiological, electrophysiological, and pathological parameters of myelin maturation were evaluated in Twitcher mice between postnatal days 20 and 45. Healthy controls showed a gradual-appearance MRI T2-weighted hypointensity of the corpus callosum (CC) starting at about P30 and ending at about P37, whereas MRI of age-matched Twitcher mice showed a complete loss of the CC-related MRI signal. MEPs allowed the functional assessment of myelin maturation within corticospinal motor pathways and showed a progressive deterioration of MEPs in Twitcher mice with increased central conduction time (CCT; 5.12 +/- 0.49 msec at P27 to 6.45 +/- 1.96 msec at P32), whereas physiological CCT shortening was found in healthy controls (3.01 +/- 0.81 msec at P27 to 2.5 +/- 0.27 msec at P32). These findings were not paralleled by traditional histological stainings. Optical observation of Bielchowsky and Luxol fast blue-PAS stainings showed mild axonal/myelin deterioration of the Twitcher brain within this time frame. Our results demonstrate that serial MRI and MEP readings are sensitive evaluation tools for in vivo monitoring of dysmyelination in Twitcher mice and underscore their potential use for longitudinal evaluation of the therapeutic impact of gene and cell therapies on these animals.
View details for DOI 10.1002/jrn.20574
View details for Web of Science ID 000230945000015
View details for PubMedID 15948181