title: An early-onset congenic strain of the motor neuron degeneration (mnd) mouse.
abstract: The mouse mutant motor neuron degeneration (mnd/mnd) has been proposed as a model of neuronal ceroid lipofuscinosis (NCL) on the basis of widespread abnormal accumulating lipopigment and neuronal and retinal degeneration. Clinically, the mutant on a C57Bl/6 genetic background shows a progressive motor abnormality starting by 6 months of age, with death prior to 12 months. When mnd is outcrossed to the AKR/J genetic background, ca. 40% of the mnd/mnd F2 progeny show early onset (onset by 4.5-5 months and death by 7 months). A congenic strain of mnd has now been produced by eight generations of backcross onto the AKR background. Mice on this background show average onset at 4 months, and most are moribund prior to 5.5 months. The early onset appears to correlate with levels of abnormal accumulating material, and should prove useful in elucidating NCL neurodegenerative mechanisms.
authors: Wadsworth Center, New York State Department of Health, Albany, New York 12201, USA.
authors: A
authors: A
authors: Messer
authors: K
authors: K
authors: Manley
authors: J A
authors: JA
authors: Plummer
mesh_terms: D000367:Age Factors; D000818:Animals; D018797:Cell Cycle Proteins; D002531:Cerebellum / Q000378:metabolism; D004195:Disease Models, Animal; D005838:Genotype; D007150:Immunohistochemistry; D051379:Mice; D020297:Mice, Congenic; D008806:Mice, Inbred AKR; D008810:Mice, Inbred C57BL; D008817:Mice, Mutant Strains; D009363:Neoplasm Proteins; D009472:Neuronal Ceroid-Lipofuscinoses / Q000235:genetics*; D011506:Proteins / Q000378:metabolism; D006180:Proton-Translocating ATPases / Q000378:metabolism
chemical_list: D018797:Cell Cycle Proteins; C103189:Fv1 protein, mouse; D009363:Neoplasm Proteins; C498808:Prcc protein, mouse; D011506:Proteins; D006180:Proton-Translocating ATPases