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Conformational disease

Nature Cell Biology volume 2, pages E207–E209 (2000)Cite this article

Abstract

A large and diverse number of diseases are now recognized as `conformational diseases', caused by adoption of non-native protein conformations that lead to aggregation. The recent conference, `Alpha1-antitrypsin deficiency and other conformational diseases', held in Airlie, Virginia, USA (27–30 June, 2000) focused on some of the common pathways by which cells protect themselves from toxicity associated with protein misfolding and aggregation.

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Figure 1: Loop–sheet polymers of α-1-antitrypsin. The severe Z-deficiency variant perturbs the structure of α-1-antitrypsin, allowing the reactive centre loop of one molecule to insert into β-sheet A of a second.
Figure 2: Inclusion bodies in different cellular compartments.

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Authors and Affiliations

  1. Department of Biological Sciences Stanford University, Stanford, 94305-5020, California, USA

    Ron R. Kopito

  2. the Skirball Institute, New York University School of Medicine, 540 First Avenue, New York, 10016, New York, USA

    David Ron

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  1. Ron R. Kopito
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  2. David Ron
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Kopito, R., Ron, D. Conformational disease. Nat Cell Biol 2, E207–E209 (2000). https://doi.org/10.1038/35041139

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