Altered native stability is the dominant basis for susceptibility of α1-antitrypsin mutants to polymerization

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

(2015) Imran Haq et al.   BIOSCIENCE REPORTS

The Roles of Helix I and Strand 5A in the Folding, Function and Misfolding of α1-Antitrypsin

(2013) Anja S. Knaupp et al.   PLoS One

Disulfide Bonding in Protein Biophysics

(2012) Deborah Fass   Annual Review of Biophysics

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

Structural Change in β-Sheet A of Z α1-Antitrypsin Is Responsible for Accelerated Polymerization and Disease

(2011) Anja S. Knaupp et al.   JOURNAL OF MOLECULAR BIOLOGY

Dynamic local unfolding in the serpin α-1 antitrypsin provides a mechanism for loop insertion and polymerization

(2011) Beena Krishnan et al.   NATURE STRUCTURAL & MOLECULAR BIOLOGY

Protein kinetic stability

(2010) Jose M. Sanchez-Ruiz   BIOPHYSICAL CHEMISTRY

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

(2010) Elena Miranda et al.   HEPATOLOGY

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

Importance of the release of strand 1C to the polymerization mechanism of inhibitory serpins

(2010) Wun-Shaing W. Chang et al.   PROTEIN SCIENCE

Conformational Pathology of the Serpins: Themes, Variations, and Therapeutic Strategies

(2009) Bibek Gooptu et al.   Annual Review of Biochemistry

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

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

Crystallographic and Cellular Characterisation of Two Mechanisms Stabilising the Native Fold of α1-Antitrypsin: Implications for Disease and Drug Design

(2009) Bibek Gooptu et al.   JOURNAL OF MOLECULAR BIOLOGY

Polymers and inflammation: disease mechanisms of the serpinopathies

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

Between-Species Variation in the Kinetic Stability of TIM Proteins Linked to Solvation-Barrier Free Energies

(2008) Miguel Costas et al.   JOURNAL OF MOLECULAR BIOLOGY

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

(2008) Masayuki Yamasaki et al.   NATURE

Dimers Initiate and Propagate Serine Protease Inhibitor Polymerisation

(2007) Aiwu Zhou et al.   JOURNAL OF MOLECULAR BIOLOGY