探討細胞自噬作用在第二型和第三型小腦脊髓運動失調症中所扮演的角色

黃靖樺

3.238.118.78，Hello！

Close
Search Quick Search Advanced Search Publication Search	Personal Service Member Login Searching History Use Bonus Card SYMSKAN
Browse Browse by University of Graduate Thesis Browse by Pinyin of Graduate Thesis Browse by Conference Proceedings Topic Browse by Conference Proceedings Publisher Browse by Pinyin of Conference Proceedings Browse by eBook Category Browse by eBook Topic Browse by eBook Publisher
Customer Services Authorize Airiti Recommend a Journal

Search Symbol (Half-width)	Description of Search Symbols
Space	"AND" indicates the intertwining of key terms used in a search
Double Quotation Marks ("") ( " " )	Double quotation marks indicate the beginning and end of a phrase, and the search will only include terms that appear in the same order of those within the quotations. Example: "image process" ： " image process "
?	Indicates a variable letter. Entering two ? will indicate two variable letters, and so on. Example: "Appl?", search results will yield apple, apply… e , appl y … ( (often used to English word searches) )
*	Indicates an unlimited number of variable letters to follow, from 1~n. Example: Enter "appl*", search results will yield apple, apples, apply, applied, application…(often used in English word searches) e , appl es , appl y , appl ied , appl ication … ( (often used to English word searches) )
AND、OR、NOT	Boolean logic combinations of key words is a skill used to expand or refine search parameters. (1) AND (1) AND: Refines search parameters (2) OR (2) OR: Expands search parameters (3) NOT: Excludes irrelevant parameters

DOI stands for Digital Object Identifier （ D igital O bject I dentifier ） ,
and is the unique identifier for objects on the internet. It can be used to create persistent link and to cite articles.

Using DOI as a persistent link

To create a persistent link, add「http://dx.doi.org/」「 http://dx.doi.org/ 」 before a DOI.
For instance, if the DOI of an article is 10.5297/ser.1201.002 , you can link persistently to the article by entering the following link in your browser: http://dx.doi.org/ 10.5297/ser.1201.002 。
The DOI link will always direct you to the most updated article page no matter how the publisher changes the document's position, avoiding errors when engaging in important research.

Cite a document with DOI

When citing references, you should also cite the DOI if the article has one. If your citation guideline does not include DOIs, you may cite the DOI link.

DOIs allow accurate citations, improve academic contents connections, and allow users to gain better experience across different platforms. Currently, there are more than 70 million DOIs registered for academic contents. If you want to understand more about DOI, please visit airiti DOI Registration （ doi.airiti.com ）。

Data Source

Health & Medical Care > Basic Medical Science
College of Medical Science and Technology > Master's Program, School of Medical Laboratory and Biotechnology

Bibliography Management Tool

Related Links

Report an Issue

Purchase Single Articles

Download PDF

探討細胞自噬作用在第二型和第三型小腦脊髓運動失調症中所扮演的角色

The Role of Autophagy in Pathogenesis of Spinalcerebellar Ataxia Type 2 and Type 3

黃靖樺 , Masters　　Advisor：蔡蕙芳　　

繁體中文

小腦脊髓運動失調症第二型；小腦脊髓運動失調症第三型；細胞自噬；細胞凋亡； Spinocerebellar ataxia type 2 and 3 ； autophagy ； apoptosis

Abstract │ Reference (97) │ Altmetrics

Abstract 〈TOP〉

Parallel Abstract 〈TOP〉

Spinocerebellar ataxia type 3 (SCA3), also known as Machado-Joseph disease (MJD), which is an autosomal dominant neurodegenerative disorders caused by expansion of CAG triplet in the coding region of ataxin-3 gene (chromosome locus 14q32.1). This CAG repeat is translated into an extended glutamine stretch in the mutant protein, which causes a gain of toxicity inducing neuronal cell death. However, the cellular mechanisms causing toxicity are still understood. In this study, two lymphoblastoid cell line (LCLs) isolated from SCA 3 patient were utilized to compare with the wide-type LCL. Our result revealed that accumulation of ataxin-3 protein were causes cell grow arrest at Sub-G1 phase, which may induce apoptosis in SCA3 cells. In the futher finding, SCA3 cells were accompanied by the loss of mitochondrial membrane potential and in relation to ROS level, there has an increased oxidative stress in SCA3 cells. In conclusion, aggregation of ataxin-3 protein can inhibited the growth of SCA3 cells via cell cycle arrest and apoptosis which may caused by MMP downregulation and increased level of ROS. Autophagy is an intracellular degradation process responsible for the clearance of most long-lived proteins and organelles. In our research used lowly concentration of bafilomycin A1 as autophagy induction. It revealed that, autophagosomal protein LC3-II (ATG8) accumulated enhances the cell viability through Bcl2 and Bax expression by western blot. The same result also shown in SCA2 cells. Inducing autophagy can decrease the toxicity which caused by accumulation of mutant ataxin-2 by detecting the proapoptotic protein, Bax expression. It suggest that up regulation of autophagy enhances the clearance of ataxin-2 and ataxin-3 proteins which may cause cell tocicity. It may be a hopeful therapeutic strategy for SCA2 and SCA3.

Reference ( 97 ) 〈TOP〉

Aguiar, J., Fernandez J., et al. Ubiquitous expression of human SCA2 gene under the regulation of the SCA2 self promoter cause specific Purkinje cell degeneration in transgenic mice.Neurosci Lett 392 (3): 202-206 , (2006).
Alonso I., Costa C., et al. A novel H101Q mutation causes PKCgamma loss in spinocerebellar ataxia type 14. J Hum Genet 50 (10): 523-529, (2005).
Andres, A. M., Lao, O., et al. Dynamics of CAG repeat loci revealed by the analysis of their variability. Hum Mutat 21 (1): 61-70, (2003).
Andrew, S. E., Goldberg, Y. P., et al. The relationship between trinucleotide (CAG) repeat length and clinical features of Huntington's disease.Nat Genet 4 (4): 398-403, (1993).
Araujo, J., P. Breuer, et al. FOXO4-dependent upregulation of superoxide dismutase-2 in response to oxidative stress is impaired in spinocerebellar ataxia type 3. Hum Mol Genet 20 (15): 2928-2941, (2011).

Altmetrics

〈TOP〉

E-mail ：

When an article is available to download, a notice will be sent to your mailbox address.

E-mail ：