twitcher mouseJoe Segen2016-11-11T15:25:48+00:00
twitcher mouse comparison of trajectory and distance covered twitcher mice to control (Wt)
A murine model with a spontaneous cerebral leukodystrophy mutation characterised by head tremors, reduced body weight, decreased activity relative to unaffected litter mates, muscle weakness in the hind limbs and progressive decline of health until death.
The twitcher phenotype has served as an experimental model for Krabbe’s disease–KD which, like KD, lacks galactosylceramidase, resulting in a toxic accumulation of fats–e.g., psychosine in the nervous system with degeneration of nerves, seizures, vision defects, and early death.
Pathology Marked reduction in myelin in the CNS and peripheral nervous system–PNS, astrocytic gliosis, multinucleated, periodic acid-Schiff-positive globoid cells and paracrystalline inclusions and twisted tubules by electron microscopy.