Korean Journal of Pediatrics 2005;48(4):425-432.
Published online April 15, 2005.
Expression of Expanded Polyglutamine Disease Proteins in Drosophila (Drosophila Polyglutamine Disease Models)
Sang Min Shin1, Kyung Hoon Paik2, Dong Kyu Jin2
1Clinical Research Center, Samsung Biomedical Research Institute, Seoul, Korea
2Department of Pediatrics, Sungkyunkwan University, School of Medicine, Samsung Medical Center, Seoul, Korea
증가된 글루타민에 의해 초래되는 뇌신경질환의 초파리 모델에 대한 연구
신상민1, 백경훈2, 진동규2
1삼성생명과학연구소
2성균관대학교 의과대학 소아과학교실
Correspondence: 
Dong Kyu Jin, Email: jindk@smc.samsung.co.kr
Abstract
Purpose
: Polyglutamine diseases are a group of diseases caused by the expansion of a polyglutamine tract in the protein. The present study was performed to verify if polyglutamine disease transgenic Drosophila models show similar dysfunctions as are seen in human patients.
Methods
: Polyglutamine disease transgenic Drosophila were tested for their climbing ability. And using genetic methods, the effects of anti-apoptotic gene bcl-2 and chemical chaperones on neurodegeneration were observed. Also, spinocerebellar ataxia 2 (SCA2) transgenic Drosophila lines were generated for future studies.
Results
: Expanded forms of spinocerebellar ataxia 3 (SCA3) transgenic protein causes characteristic locomotor dysfunction when expressed in the nervous system of Drosophila but the anti-apoptotic gene bcl-2 shows no evidence of ameliorating the deleterious effect of the expanded protein. However, Glycerol, a chemical chaperone, seemed to reduce the toxicity, at least in the eyes of the transgenic flies. The level SCA2 expression is too weak in the transgenic SCA2 Drosophila for evaluation.
Conclusion
: SCA3 transgenic Drosophila show ataxic behavior as observed in human patients. Chemical chaperones such as glycerol may prove beneficial in this class of genetic disease, which has no current method of cure.
Key Words: Polyglutamine , Machdo-Joseph disease (MJD) , Spinocerebellar ataxia 3 (SCA3) , Drosophila , Behavioral dysfunction , bcl-2 , Spinocerebellar ataxia 2 (SCA2) , Chaperones


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