Glutamine repeats as polar zippers: their possible role in inherited neurodegenerative diseases.

• M F Perutz

  Medical Research Council Laboratory of Molecular Biology, Cambridge, England.

• T Johnson

  Medical Research Council Laboratory of Molecular Biology, Cambridge, England.

• M Suzuki

  Medical Research Council Laboratory of Molecular Biology, Cambridge, England.

• J T Finch

  Medical Research Council Laboratory of Molecular Biology, Cambridge, England.

抄録

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.

収録刊行物

• Proceedings of the National Academy of Sciences

  Proceedings of the National Academy of Sciences 91 (12), 5355-5358, 1994-06-07

  Proceedings of the National Academy of Sciences

キーワード

• Multidisciplinary

詳細情報

• CRID: 1360011146582299008
• NII論文ID: 30016296538
• DOI: 10.1073/pnas.91.12.5355
• ISSN: 10916490
• ISSN: 00278424
• Web Site: https://pnas.org/doi/pdf/10.1073/pnas.91.12.5355
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