| 摘要 |
The instant disclosure describes an electrophoretic procedure capable of resolving and isolating ultra high molecular weight (MW) protein aggregates from nerve tissue. The procedure is based on the use of composite agarose-polyacrylamide gel electrophoresis (CAPAGE) and resolves proteins and protein aggregates over the range of from approximately 225 kDa to approximately 30,000 kDa. Triton X-100 precipitation is used to obtain a cytoskeleton protein fraction that is subsequently resuspended and subjected to gel electrophoresis. This method demonstrates that a protein aggregate of approximately 30,000 kDa is characteristic of normal murine spinal cord tissue and that the amount of said protein aggregate is increased in spinal cord homogenate obtained from transgenic mice bearing copies of a mutant human gene characteristic of familial amyotrophic lateral sclerosis. This method for separating nerve tissue ultra high MW cytoskeleton protein aggregates can prove useful in a variety of future biophysical and pharmacological studies related to the etiologies of Charcot-Marie-Tooth disease, Alzheimer's disease, Parkinson's disease, diseases based on expansions in tandem DNA repeats, spinal muscular atrophy, Friedreich's ataxia, giant axon neuropathy, juvenile ceroid-lipofuscinosis, amyotrophic lateral sclerosis, diabetic polyneuropathy and Down's syndrome.
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