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Amyotrophic lateral sclerosis (ALS) is a progressive, fatal disease characterized by a selective degeneration of motor neurons in the spinal cord, brain stem and motor cortex. Over-expressing the human mutant Gly93®Ala Superoxide dismutase 1 protein (G93A-SOD1) in mice causes a phenotype characterized by degenerating motor neurons.
Collaboration: Dr. Jürgen Goldschmidt (IfN), PD Dr. S. Vielhaber (Department of Neurology II, OvGU), Prof. Dr. A. C. Ludolph (Department of Neurology, University of Ulm), Prof. D. Leibfritz (Department of Organic Chemistry, University of Bremen)
Fig. 1. T2-weighted MR-images of mice over-expressing the human mutant G93A-SOD. At age of 130d a clear increase in signal intensity could be observed in the hypoglossal nucleus (Nc. Nv. XII; arrow top row left), the nucleus of the solitary tract (arrow top row middle), and the trigeminal nucleus, the facial nucleus, and the nucleus ambiguus (Nc. Nv. V, VII and motoric part of Nc Nv. IX, X, arrows top row left). These nuclei are not detectable in an identical T2- weighted image in aged-matched litter-mates, that do not express the G93A-SOD (lower row). Section thickness 600 µm, field of view 30x30 mm, matrix 256x256 (calculated in plane resolution 117 µm).
Fig. 2. The signal intensity of motoric nuclei within the brain stem changes over time. Sequential T2-weighted MR-images of G93A-SOD mice were obtained at day 50, 90, 112, and 130. The left side shows representative sagital sections at various time-points, which are used to measure the signal intensity changes of the hypoglossal nucleus. The signal intensities of the hypoglossal nucleus (Nc. Nv. XII), facial nucleus (Nc. Nv. VII), trigeminal nucleus (Nc. Nv. V) were measured and calculated as ratio to the surrounding brain stem tissue (right side). Whereas at day 50 no motoric nuclei were detectable by T2-weighted MRI, the signal intensities of all measured nuclei increases over time.