GLUTAMINE REPEATS AS POLAR ZIPPERS - THEIR POSSIBLE ROLE IN INHERITED NEURODEGENERATIVE DISEASES

被引：911

作者：

PERUTZ, MF

JOHNSON, T

SUZUKI, M

FINCH, JT

机构：

[1] Med. Res. Cncl. Lab. of Molec. Biol.

来源：

PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA | 1994年 / 91卷 / 12期

关键词：

D O I：

10.1073/pnas.91.12.5355

中图分类号：

O [数理科学和化学]; P [天文学、地球科学]; Q [生物科学]; N [自然科学总论];

学科分类号：

07 ; 0710 ; 09 ;

摘要：

Four inherited neurodegenerative diseases are linked to abnormally expanded repeats of glutamine residues in the affected proteins. Molecular modeling followed by optical, electron, and x-ray diffraction studies of a synthetic poly(L-glutamine) shows that it forms beta-sheets strongly held together by hydrogen bonds. Glutamine repeats may function as polar zippers, for example, by joining specific transcription factors bound to separate DNA segments. Their extension may cause disease either by increased, nonspecific affinity between such factors or by gradual precipitation of the affected proteins in neurons.

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页码：5355 / 5358

页数：4

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