Intramolecular i-motif structure at acidic pH for Progressive Myoclonus Epilepsy (EPM1) repeat d(CCCCGCCCCGCG)n

被引:24
|
作者
Pataskar, SS
Dash, D
Brahmachari, SK
机构
[1] CSIR, Ctr BIochem Technol, Funct Genom Unit, Delhi 110007, India
[2] Indian Inst Sci, Mol Biophys Unit, Bangalore 560012, Karnataka, India
来源
关键词
D O I
10.1080/07391102.2001.10506741
中图分类号
Q5 [生物化学]; Q7 [分子生物学];
学科分类号
071010 ; 081704 ;
摘要
The most common mutation associated with Progressive Myoclonus Epilepsy (EPM1) of Unverricht-Lundberg type is the expansion of a dodecamer repeat, d(CCCCGCCCCGCG)n. We show that the C-rich strand of this repeat (2-3 copies) forms intercalated i-motif structure at acidic pH as judged by CD spectroscopy and anomalous gel electrophoretic mobility. The stability of the structure increases with the increase in the length of the repeat. Transient formation of stable, folded back structure like i-motif could play an important role in the mechanism of expansion of this repeat.
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页码:307 / 313
页数:7
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