| 1 |
35394846 | Z-α1-antitrypsin polymers impose molecular filtration in the endoplasmic reticulum after undergoing phase transition to a solid state. | 2022 Apr 8 |
1 |
| 2 |
33058391 | The molecular species responsible for α1 -antitrypsin deficiency are suppressed by a small molecule chaperone. | 2021 Apr |
1 |
| 3 |
34063488 | Scaling Concepts in Serpin Polymer Physics. | 2021 May 15 |
1 |
| 4 |
28504839 | The pathological Trento variant of alpha-1-antitrypsin (E75V) shows nonclassical behaviour during polymerization. | 2017 Jul |
1 |
| 5 |
28752464 | Electrophoresis- and FRET-Based Measures of Serpin Polymerization. | 2017 |
1 |
| 6 |
27601439 | The endoplasmic reticulum remains functionally connected by vesicular transport after its fragmentation in cells expressing Z-α1-antitrypsin. | 2016 Dec |
1 |
| 7 |
25462157 | Characterising the association of latency with α(1)-antitrypsin polymerisation using a novel monoclonal antibody. | 2015 Jan |
1 |
| 8 |
25757566 | A single-chain variable fragment intrabody prevents intracellular polymerization of Z α1-antitrypsin while allowing its antiproteinase activity. | 2015 Jun |
1 |
| 9 |
24374162 | The molecular and cellular pathology of α₁-antitrypsin deficiency. | 2014 Feb |
1 |
| 10 |
24603821 | Circulating polymers in α1-antitrypsin deficiency. | 2014 May |
1 |
| 11 |
25418171 | The shapes of Z-α1-antitrypsin polymers in solution support the C-terminal domain-swap mechanism of polymerization. | 2014 Oct 21 |
2 |
| 12 |
25442121 | Molecular pathogenesis of alpha-1-antitrypsin deficiency. | 2014 Dec |
1 |
| 13 |
23197448 | Endoplasmic reticulum polymers impair luminal protein mobility and sensitize to cellular stress in alpha1-antitrypsin deficiency. | 2013 May |
3 |
| 14 |
23382962 | The roles of helix I and strand 5A in the folding, function and misfolding of α1-antitrypsin. | 2013 |
1 |
| 15 |
23527623 | Twenty years of polymers: a personal perspective on alpha-1 antitrypsin deficiency. | 2013 Mar |
2 |
| 16 |
22607686 | Encapsulation of alpha-1 antitrypsin in PLGA nanoparticles: in vitro characterization as an effective aerosol formulation in pulmonary diseases. | 2012 May 20 |
1 |
| 17 |
21498872 | Gene therapy for alpha-1 antitrypsin deficiency. | 2011 Apr 15 |
2 |
| 18 |
21765645 | Effects of glycosylation on the stability and flexibility of a metastable protein: the human serpin α(1)-antitrypsin. | 2011 Apr |
1 |
| 19 |
21961602 | Local conformational flexibility provides a basis for facile polymer formation in human neuroserpin. | 2011 Oct 5 |
1 |
| 20 |
20583215 | A novel monoclonal antibody to characterize pathogenic polymers in liver disease associated with alpha1-antitrypsin deficiency. | 2010 Sep |
1 |
| 21 |
19218787 | New Findings in PiZZ alpha1-antitrypsin deficiency-related panniculitis. Demonstration of skin polymers and high dosing requirements of intravenous augmentation therapy. | 2009 |
3 |
| 22 |
19245336 | Conformational pathology of the serpins: themes, variations, and therapeutic strategies. | 2009 |
2 |
| 23 |
19426146 | alpha1-Antitrypsin deficiency, chronic obstructive pulmonary disease and the serpinopathies. | 2009 May 14 |
1 |
| 24 |
19437508 | Molecular characterization of the new defective P(brescia) alpha1-antitrypsin allele. | 2009 Aug |
2 |
| 25 |
18794298 | The structural basis of serpin polymerization studied by hydrogen/deuterium exchange and mass spectrometry. | 2008 Nov 7 |
1 |
| 26 |
17918823 | Small molecules block the polymerization of Z alpha1-antitrypsin and increase the clearance of intracellular aggregates. | 2007 Nov 1 |
1 |
| 27 |
19164889 | Protein misfolding and the serpinopathies. | 2007 Jan-Mar |
1 |
| 28 |
16439713 | The selective advantage of alpha1-antitrypsin deficiency. | 2006 May 15 |
1 |
| 29 |
16704419 | Sugar and alcohol molecules provide a therapeutic strategy for the serpinopathies that cause dementia and cirrhosis. | 2006 Jun |
1 |
| 30 |
16921127 | Parker B. Francis lectureship. Antitrypsin deficiency, the serpinopathies, and chronic obstructive pulmonary disease. | 2006 Aug |
1 |
| 31 |
15787598 | Molecular mousetraps and the serpinopathies. | 2005 Apr |
1 |
| 32 |
15170041 | Alpha1-antitrypsin deficiency. 4: Molecular pathophysiology. | 2004 Jun |
1 |
| 33 |
15486938 | Differential detection of PAS-positive inclusions formed by the Z, Siiyama, and Mmalton variants of alpha1-antitrypsin. | 2004 Nov |
2 |
| 34 |
12588861 | Drosophila necrotic mutations mirror disease-associated variants of human serpins. | 2003 Apr |
1 |
| 35 |
12731467 | [From gene disease; alpha1-antitrypsine deficiency]. | 2003 Apr 19 |
1 |
| 36 |
12034572 | Polymers of alpha(1)-antitrypsin are chemotactic for human neutrophils: a new paradigm for the pathogenesis of emphysema. | 2002 Jun |
1 |
| 37 |
11767949 | Osmolytes as modulators of conformational changes in serpins. | 2001 Nov |
1 |
| 38 |
10092631 | Probing the unfolding pathway of alpha1-antitrypsin. | 1999 Apr 2 |
1 |
| 39 |
10536068 | Serine proteinase inhibitor therapy in alpha(1)-antitrypsin inhibitor deficiency and cystic fibrosis. | 1999 Nov |
1 |
| 40 |
10543942 | A 2.6 A structure of a serpin polymer and implications for conformational disease. | 1999 Oct 29 |
1 |
| 41 |
9466920 | Wild-type alpha 1-antitrypsin is in the canonical inhibitory conformation. | 1998 Jan 23 |
1 |
| 42 |
9569237 | Lung polymers in Z alpha1-antitrypsin deficiency-related emphysema. | 1998 May |
4 |
| 43 |
9293373 | Albumin contamination of a purified human alpha 1-antitrypsin preparation does not affect either structural conformation or the electrophoretic mobility of the inhibitor. | 1997 Aug 29 |
1 |
| 44 |
8922028 | Computer modelling of human alpha 1-antitrypsin reactive site loop behaviour under mild conditions. | 1996 |
1 |
| 45 |
7506655 | Plasminogen-activator inhibitor type 2 (PAI-2) is a spontaneously polymerising SERPIN. Biochemical characterisation of the recombinant intracellular and extracellular forms. | 1993 Dec 15 |
1 |
| 46 |
8464163 | [Genetic analyses of alpha 1-antitrypsin deficiency]. | 1993 Feb |
1 |