Ubiquitin deposits are present in spinal motor neurons in all stages of the disease in the motor neuron degeneration (Mnd) mutant of the mouse.
Monoclonal antibodies to ubiquitin were used in an immunocytochemical analysis of spinal cord from the Mnd (motor neuron degeneration) mouse, an animal model for motor neuron disease. Tissue from mice with mild, moderate and severe disease, from presymptomatic mice, and age-matched controls were analyzed. Ubiquitin deposits were observed in spinal neurons from presymptomatic animals and all stages of the disease. No immunoreactive deposits were observed in control mice at the concentration of the antibodies used. The presence of ubiquitin immunopositivity in presymptomatic spinal motor neurons suggests that ubiquitination might play a primary role in the pathogenesis of motor neuron disease in the Mnd mouse, and perhaps of motor neuron disease in general.
Mazurkiewicz
J E
JE
Department of Anatomy, Cell Biology and Neurobiology, Albany Medical College, NY 12208.
D000818:Animals; D000911:Antibodies, Monoclonal; D003593:Cytoplasm / Q000378:metabolism; D007150:Immunohistochemistry; D051379:Mice; D008810:Mice, Inbred C57BL; D008818:Mice, Neurologic Mutants; D009046:Motor Neurons / Q000378:metabolism*; D009410:Nerve Degeneration / Q000502:physiology*; D013116:Spinal Cord / Q000033:anatomy & histology / Q000166:cytology / Q000378:metabolism*; D014452:Ubiquitins / Q000378:metabolism*
D000911:Antibodies, Monoclonal; D014452:Ubiquitins