[The mouse mutant "Jimpy". Animal model of leukodystrophy].
The Jimpy mouse is an x-linked recessive mutation of the house mouse resulting a myelin deficiency in the central nervous system. It is of interest as an animal model of human leukodystrophy. Clinical symptoms include ataxia  and tremor as initial signs, followed by tonic-clonic convulsions prefinally. The myelin deficiency is probably caused by a developmental disturbance of the oligodendrogial cell line. Clinical and pathological findings in the mouse mutant Jimpy show some remarkable similarities to Pelizaeus-Merzbacher disease.
D000818:Animals; D000831:Animals, Newborn; D004195:Disease Models, Animal; D006720:Homozygote; D007966:Leukodystrophy, Metachromatic / Q000235:genetics*; D008052:Lipid Metabolism, Inborn Errors; D051379:Mice; D009186:Myelin Sheath; D009457:Neuroglia / Q000002:abnormalities; D009900:Optic Nerve / Q000648:ultrastructure
Die Maus-Mutante "Jimpy".