Article
Unravelling the twists and turns of the serpinopathies.
The FEBS journal - 1 Oct 2011
Roussel Benoit D, Irving James A, Ekeowa Ugo I, Belorgey Didier, Haq Imran, Ordóñez Adriana, Kruppa Antonina J, Duvoix Annelyse, Rashid Sheikh Tamir, Crowther Damian C, Marciniak Stefan J, Lomas David A
Abstract excerpt
Members of the serine protease inhibitor (serpin) superfamily are found in all branches of life and play an important role in the regulation of enzymes involved in proteolytic cascades. Mutants of the serpins result in a delay in folding, with unstable intermediates being cleared by endoplasmic reticulum-associated degradation. The remaining protein is either fully folded and secreted or retained as ordered...
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