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Benoit D. Roussel, James A. Irving, Ugo I. Ekeowa, Didier Belorgey, Imran Haq, Adriana Ordóñez, Antonina J. Kruppa, Annelyse Duvoix, Sheikh Tamir Rashid, Damian C. Crowther, Stefan J. Marciniak and David A. Lomas Unravelling the twists and turns of the serpinopathies FEBS Journal 278

Article first published online: 20 JUN 2011 | DOI: 10.1111/j.1742-4658.2011.08201.x

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Mutants of the serpins form ordered polymers that result in a group of diseases termed the serpinopathies. These are typified by mutations in α1-antitrypsin to cause cirrhosis and in neuroserpin to cause the dementia FENIB. We review the pathobiology of the serpinopathies and the development of novel therapeutic strategies to treat the inclusions that cause disease

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