Reactive centre loop mutants of α-1-antitrypsin reveal position-specific effects on intermediate formation along the polymerization pathway

Structural Dynamics Associated with Intermediate Formation in an Archetypal Conformational Disease

(2012) Mun Peak Nyon et al.   STRUCTURE

Molecular basis of α1-antitrypsin deficiency revealed by the structure of a domain-swapped trimer

(2011) Masayuki Yamasaki et al.   EMBO REPORTS

Molecular contortionism – on the physical limits of serpin ‘loop-sheet’ polymers

(2010) James A. Huntington et al.   BIOLOGICAL CHEMISTRY

A novel monoclonal antibody to characterize pathogenic polymers in liver disease associated with α1-antitrypsin deficiency

(2010) Elena Miranda et al.   HEPATOLOGY

Loop-Sheet Mechanism of Serpin Polymerization Tested by Reactive Center Loop Mutations

(2010) Masayuki Yamasaki et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Characterisation of serpin polymers in vitro and in vivo

(2010) Didier Belorgey et al.   METHODS

Defining the mechanism of polymerization in the serpinopathies

(2010) U. I. Ekeowa et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Cleaved antitrypsin polymers at atomic resolution

(2010) Michelle A. Dunstone et al.   PROTEIN SCIENCE

α1-Antitrypsin deficiency, chronic obstructive pulmonary disease and the serpinopathies

(2009) Ugo I. Ekeowa et al.   CLINICAL SCIENCE

Endoplasmic Reticulum-associated Degradation (ERAD) and Autophagy Cooperate to Degrade Polymerogenic Mutant Serpins

(2009) Heike Kroeger et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Kinetic Instability of the Serpin Z α1-Antitrypsin Promotes Aggregation

(2009) Anja S. Knaupp et al.   JOURNAL OF MOLECULAR BIOLOGY

Polymers and inflammation: disease mechanisms of the serpinopathies

(2008) Bibek Gooptu et al.   JOURNAL OF EXPERIMENTAL MEDICINE

Crystal structure of a stable dimer reveals the molecular basis of serpin polymerization

(2008) Masayuki Yamasaki et al.   NATURE