Warning: Undefined array key "mm" in /www/wwwroot/www.ai-bt.com/si.php on line 10 Deprecated: trim(): Passing null to parameter #1 ($string) of type string is deprecated in /www/wwwroot/www.ai-bt.com/si.php on line 10 Alpha1-antitrypsin polymerization and the serpinopathies: pathobiology and prospects for therapy.

× No keyword cloud information.

32 in total

1. Inactive conformation of the serpin alpha(1)-antichymotrypsin indicates two-stage insertion of the reactive loop: implications for inhibitory function and conformational disease.

Authors: B Gooptu; B Hazes; W S Chang; T R Dafforn; R W Carrell; R J Read; D A Lomas
Journal: Proc Natl Acad Sci U S A Date: 2000-01-04 Impact factor: 11.205

2. Topography of a 2.0 A structure of alpha1-antitrypsin reveals targets for rational drug design to prevent conformational disease.

Authors: P R Elliott; X Y Pei; T R Dafforn; D A Lomas
Journal: Protein Sci Date: 2000-07 Impact factor: 6.725

3. 6-mer peptide selectively anneals to a pathogenic serpin conformation and blocks polymerization. Implications for the prevention of Z alpha(1)-antitrypsin-related cirrhosis.

Authors: Ravi Mahadeva; Timothy R Dafforn; Robin W Carrell; David A Lomas
Journal: J Biol Chem Date: 2001-12-28 Impact factor: 5.157

Review 4. Alpha1-antitrypsin deficiency--a model for conformational diseases.

Authors: Robin W Carrell; David A Lomas
Journal: N Engl J Med Date: 2002-01-03 Impact factor: 91.245

Review 5. Dissecting glycoprotein quality control in the secretory pathway.

Authors: C M Cabral; Y Liu; R N Sifers
Journal: Trends Biochem Sci Date: 2001-10 Impact factor: 13.807

6. Mutant Neuroserpin (S49P) that causes familial encephalopathy with neuroserpin inclusion bodies is a poor proteinase inhibitor and readily forms polymers in vitro.

Authors: Didier Belorgey; Damian C Crowther; Ravi Mahadeva; David A Lomas
Journal: J Biol Chem Date: 2002-03-05 Impact factor: 5.157

7. Prevention of polymerization of M and Z alpha1-Antitrypsin (alpha1-AT) with trimethylamine N-oxide. Implications for the treatment of alpha1-at deficiency.

Authors: G L Devlin; H Parfrey; D J Tew; D A Lomas; S P Bottomley
Journal: Am J Respir Cell Mol Biol Date: 2001-06 Impact factor: 6.914

Review 8. Evolving concepts in the pathogenesis of chronic obstructive pulmonary disease.

Authors: S D Shapiro
Journal: Clin Chest Med Date: 2000-12 Impact factor: 2.878

9. The proteasome participates in degradation of mutant alpha 1-antitrypsin Z in the endoplasmic reticulum of hepatoma-derived hepatocytes.

Authors: J H Teckman; J Burrows; T Hidvegi; B Schmidt; P D Hale; D H Perlmutter
Journal: J Biol Chem Date: 2001-09-27 Impact factor: 5.157

10. Chemotactic activity of elastin-derived peptides.

Authors: R M Senior; G L Griffin; R P Mecham
Journal: J Clin Invest Date: 1980-10 Impact factor: 14.808

87 in total

Review 1. Protein aggregation in disease: a role for folding intermediates forming specific multimeric interactions.

Authors: Arthur Horwich
Journal: J Clin Invest Date: 2002-11 Impact factor: 14.808

2. The cellular response to aggregated proteins associated with human disease.

Authors: David H Perlmutter
Journal: J Clin Invest Date: 2002-11 Impact factor: 14.808

Review 3. Liver injury in alpha1-antitrypsin deficiency: an aggregated protein induces mitochondrial injury.

Authors: David H Perlmutter
Journal: J Clin Invest Date: 2002-12 Impact factor: 14.808

Review 4. Orchestrating the unfolded protein response in health and disease.

Authors: Randal J Kaufman
Journal: J Clin Invest Date: 2002-11 Impact factor: 14.808

Review 5. Matrix metalloproteinases, a disintegrin and metalloproteinases, and a disintegrin and metalloproteinases with thrombospondin motifs in non-neoplastic diseases.

Authors: Takayuki Shiomi; Vincent Lemaître; Jeanine D'Armiento; Yasunori Okada
Journal: Pathol Int Date: 2010-07 Impact factor: 2.534

6. Dominant-negative SERPING1 variants cause intracellular retention of C1 inhibitor in hereditary angioedema.

Authors: Didde Haslund; Laura Barrett Ryø; Sara Seidelin Majidi; Iben Rose; Kristian Alsbjerg Skipper; Tue Fryland; Anja Bille Bohn; Claus Koch; Martin K Thomsen; Yaseelan Palarasah; Thomas J Corydon; Anette Bygum; Lene N Nejsum; Jacob Giehm Mikkelsen
Journal: J Clin Invest Date: 2018-12-10 Impact factor: 14.808

7. Mutant fibrinogen cleared from the endoplasmic reticulum via endoplasmic reticulum-associated protein degradation and autophagy: an explanation for liver disease.

Authors: Kristina B Kruse; Amy Dear; Erin R Kaltenbrun; Brandan E Crum; Peter M George; Stephen O Brennan; Ardythe A McCracken
Journal: Am J Pathol Date: 2006-04 Impact factor: 4.307

Review 8. Protein misfolding and the serpinopathies.

Authors: Didier Belorgey; Peter Hägglöf; Susanna Karlsson-Li; David A Lomas
Journal: Prion Date: 2007-01-06 Impact factor: 3.931

Review 9. Serpins, immunity and autoimmunity: old molecules, new functions.

Authors: Mariele Gatto; Luca Iaccarino; Anna Ghirardello; Nicola Bassi; Patrizia Pontisso; Leonardo Punzi; Yehuda Shoenfeld; Andrea Doria
Journal: Clin Rev Allergy Immunol Date: 2013-10 Impact factor: 8.667

10. pH-dependent stability of neuroserpin is mediated by histidines 119 and 138; implications for the control of beta-sheet A and polymerization.

Authors: Didier Belorgey; Peter Hägglöf; Maki Onda; David A Lomas
Journal: Protein Sci Date: 2010-02 Impact factor: 6.725