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학술연구/단체지원/교육 등 연구자 활동을 지속하도록 DBpia가 지원하고 있어요.
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논문 기본 정보
- 자료유형
- 학술저널
- 저자정보
- 발행연도
- 2017.1
- 수록면
- 321 - 328 (8page)
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초록· 키워드
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Huntington disease (HD) is an inherited neurodegenerative disorder characterized by motor and cognitive dysfunction caused by expansion of polyglutamine (polyQ) repeat in exon 1 of huntingtin (HTT). In patients, the number of glutamine residues in polyQ tracts are over 35, and it is correlated with age of onset, severity, and disease progression. Expansion of polyQ increases the propensity for HTT protein aggregation, process known to be implicated in neurodegeneration. These pathological aggregates can be transmitted from neuron to another neuron, and this process may explain the pathological spreading of polyQ aggregates. Here, we developed an in vivo model for studying transmission of polyQ aggregates in a highly quantitative manner in real time. HTT exon 1 with expanded polyQ was fused with either N-terminal or C-terminal fragments of Venus fluorescence protein and expressed in pharyngeal muscles and associated neurons, respectively, of C. elegans. Transmission of polyQ proteins was detected using bimolecular fluorescence complementation (BiFC). Mutant polyQ (Q97) was transmitted much more efficiently than wild type polyQ (Q25) and forms numerous inclusion bodies as well. The transmission of Q97 was gradually increased with aging of animal. The animals with polyQ transmission exhibited degenerative phenotypes, such as nerve degeneration, impaired pharyngeal pumping behavior, and reduced life span. The C. elegans model presented here would be a useful in vivo model system for the study of polyQ aggregate propagation and might be applied to the screening of genetic and chemical modifiers of the propagation.
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참고문헌 (36)
참고문헌 신청
Li XJ / 2011 / Li S(2011)Proteasomal dysfunction in aging and Huntington disease / Neurobiol Dis 43:4~8
Perluigi M / 2005 / Proteomic analysis of protein expression and oxidative modification in r6/2 transgenic mice: a model of Huntington disease / Mol Cell Proteomics 4:1849~1861
Stoy N / 2005 / Tryptophan metabolism and oxidative stress in patients with Huntington’s disease / J Neurochem 93:611~623
Holmberg CI / 2004 / Inefficient degradation of truncated polyglutamine proteins by the proteasome / EMBO J 23:4307~4318
Waelter S / 2001 / Accumulation of mutant huntingtin fragments in aggresome-like inclusion bodies as a result of insufficient protein degradation / Mol Biol Cell 12:1393~1407
Perluigi M / 2005 / Proteomic analysis of protein expression and oxidative modification in r6/2 transgenic mice: a model of Huntington disease / Mol Cell Proteomics 4:1849~1861
Stoy N / 2005 / Tryptophan metabolism and oxidative stress in patients with Huntington’s disease / J Neurochem 93:611~623
Holmberg CI / 2004 / Inefficient degradation of truncated polyglutamine proteins by the proteasome / EMBO J 23:4307~4318
Waelter S / 2001 / Accumulation of mutant huntingtin fragments in aggresome-like inclusion bodies as a result of insufficient protein degradation / Mol Biol Cell 12:1393~1407
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