경직성 뇌성마비 환자에서 근육내 Ubiquitin 및 Neural Cell Adhesion Molecule 의 발현

Abstract

Purpose: This study was performed to examine the histopathologic changes of muscles and the expression patterns of ubiquitin and N-CAM (neural cell adhesion molecule) in accordance with cerebral palsy patient’s spasticity.

Materials and Methods: We studied thirteen specimens from seven patients with spastic cerebral palsy, five patients suspected to have neuromuscular diseases, and one normal person. We performed the routine histologic procedures, the reverse transcriptional polymerase chain reaction (RT-PCR), and immunostaining.

Results: There were no disease-specific abnormalities related with the degree of spasticity on histopathologic evaluation. However, in the cerebral palsy patients, the degree of spasticity seems to have positive correlations with the expression of ubiquitin gene and negative correlations with the expression of N-CAM gene. On the other hand, in the immunostaining procedures, the reactions to ubiquitin protein were all negative and reactions to N-CAM protein were strongly positive only in two hereditary motor sensory neuropathy patients.

Conclusion: The results of our study seem to be caused by multiple mechanisms. If more studies about the changes after the transcription of ubiquitin and N-CAM genes are performed, these results can be applied to the research and treatment of cerebral palsy on molecular biologic aspects.