Publications

Latest publication 02/27/2008

Neuron-Specific Expression of Mutant Superoxide Dismutase Is Sufficient to Induc

Mutations in superoxide dismutase (SOD1) cause amyotrophic lateral sclerosis (ALS), an adult-onset progressive paralytic disease characterized by...

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    [title] => Neuron-Specific Expression of Mutant Superoxide Dismutase Is Sufficient to Induc
    [paragraph] => Neuron-Specific Expression of Mutant Superoxide Dismutase Is Sufficient to Induce Amyotrophic Lateral Sclerosis in Transgenic Mice.
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Authors
D. Jaarsma, E. Teuling, E. Haasdijk, C. De Zeeuw, C. Hoogenraad.

 Lab
Erasmus Medical Center, Department of Neuroscience, Rotterdam, The Netherlands.
Journal
The Journal of Neuroscience
Abstract
Mutations in superoxide dismutase (SOD1) cause amyotrophic lateral sclerosis (ALS), an adult-onset progressive paralytic disease characterized by loss of motor neurons, and cause an ALS-like disease when expressed in mice. Recent data have suggested that motor neuron degeneration results from toxic actions of mutant SOD1 operating in both motor neurons and their neighboring glia, raising the question whether mutant SOD1 expression selectively in neurons is sufficient to induce disease. Here we show that neuronal expression of mutant SOD1 is sufficient to cause motor neuron degeneration and paralysis in transgenic mice with cytosolic dendritic ubiquitinated SOD1 aggregates as the dominant pathological feature. In addition, we show that crossing our neuron-specific mutant SOD1 mice with ubiquitously wild-type SOD1-expressing mice leads to dramatic wild-type SOD1 aggregation in oligodendroglia after the onset of neuronal degeneration. Together, our findings support a pathogenic scenario in which mutant SOD1 in neurons triggers neuronal degeneration, which in turn may facilitate aggregate formation in surrounding glial cells.
BIOSEB Instruments Used
Grip strength test (BIO-GS3)

Keywords/Topics
Amyotrophic Lateral Sclerosis (ALS); Phenotyping; Muscular system; Cross-disciplinary subjects 
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An easy way to objectively quantify the muscular strength of mice and rats, and to assess the effect of drugs, toxins, muscular (i.e. myopathy) and neurodegenerative diseases on muscular degeneration. It is widely used in conjunction with the ROTAROD motor coordination test: a normally coordinated rodent will show a decreased latency to fall off the rotating rod if its muscular strength is low. The Grip Strength Test is a must for your research on activity, motor control & coordination, and is particularly well suited for studies on Parkinson's & Huntington's disease.
New features GS4 - 2023: Color display with permanent backlight screen for easier reading, reset by footswitch, Improved battery time, Larger data memory of 500 values, Animal counter, USB port (charging/data transfer)

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