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Published on: January 7, 2019
Neurofilament distribution is altered in the Mnd (motor neuron degeneration) mouse
L M Callahan1, E L Wylen, A Messer
1Department of Anatomy, Cell Biology and Neurobiology, Albany Medical College, NY 12208.
Abstract:
Motor neuron degeneration (Mnd) is a genetic neurodegenerative disease of the mouse that is characterized by a progressive increase in motor dysfunction, moving from hind to fore limbs, leading to paralysis. An immunocytochemical analysis of the neurofilament distribution in spinal motor neurons in Mnd mice from all stages of the disease, including the presymptomatic, was performed using antibodies to different neurofilament subunits with different degrees of phosphorylation. Perikarya that stained with antibodies to phosphorylated neurofilaments were present in Mnd and control spinal cords, but the number of stained perikarya in Mnd was not significantly different from controls. There was a marked redistribution of neurofilaments within the cytoplasm of some motor neurons in Mnd cords. In Mnd but not controls, the immunoreaction product appeared marginated, leaving areas in the cytoplasm absent of immunostaining. These areas were observed in all stages of the disease, but less predictably in presymptomatics. Both the size of the areas and the number of motoneurons containing these areas appeared to increase with the severity of the disease. The number of anterior horn neurons in the hind limb region of lamina IX in spinal segment L4 of Mnd was lower than in controls, suggesting there is a loss of neurons in Mnd.
Insights
Motor neuron degeneration (Mnd) in mice shows abnormal neurofilament distribution within motor neurons, with marginated staining appearing as the disease progresses. This suggests a potential link between neurofilament changes and motor neuron loss in this genetic neurodegenerative condition.
Area of Science:
- Neuroscience
- Genetics
- Pathology
Background:
- Motor neuron degeneration (Mnd) is a genetic neurodegenerative disease in mice.
- It is characterized by progressive motor dysfunction, leading to paralysis.
Purpose of the Study:
- To investigate neurofilament distribution in spinal motor neurons of Mnd mice.
- To correlate neurofilament changes with disease progression and neuron loss.
Main Methods:
- Immunocytochemical analysis using antibodies against phosphorylated neurofilament subunits.
- Examination of spinal motor neurons at various disease stages, including presymptomatic.
- Quantitative assessment of neurofilament distribution and neuron counts.
Main Results:
- A marked redistribution of neurofilaments was observed in Mnd motor neurons, with marginated immunostaining.
- Areas devoid of immunostaining within the cytoplasm increased in size and prevalence with disease severity.
- A reduction in anterior horn neuron numbers was noted in Mnd mice, particularly in the hind limb region.
Conclusions:
- Abnormal neurofilament redistribution is a key feature of motor neuron degeneration in Mnd mice.
- These cytoskeletal changes correlate with disease progression and may contribute to motor neuron loss.
