Literature DB >> 21921939

The structural diversity in α1-antitrypsin misfolding.

Stephen P Bottomley1.   

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Year:  2011        PMID: 21921939      PMCID: PMC3185355          DOI: 10.1038/embor.2011.187

Source DB:  PubMed          Journal:  EMBO Rep        ISSN: 1469-221X            Impact factor:   8.807


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  8 in total

1.  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

2.  Defining the mechanism of polymerization in the serpinopathies.

Authors:  Ugo I Ekeowa; Joanna Freeke; Elena Miranda; Bibek Gooptu; Matthew F Bush; Juan Pérez; Jeff Teckman; Carol V Robinson; David A Lomas
Journal:  Proc Natl Acad Sci U S A       Date:  2010-09-20       Impact factor: 11.205

3.  The mechanism of Z alpha 1-antitrypsin accumulation in the liver.

Authors:  D A Lomas; D L Evans; J T Finch; R W Carrell
Journal:  Nature       Date:  1992-06-18       Impact factor: 49.962

4.  Cirrhosis associated with alpha-1-antitrypsin deficiency: a previously unrecognized inherited disorder.

Authors:  H L Sharp; R A Bridges; W Krivit; E F Freier
Journal:  J Lab Clin Med       Date:  1969-06

5.  A novel monoclonal antibody to characterize pathogenic polymers in liver disease associated with alpha1-antitrypsin deficiency.

Authors:  Elena Miranda; Juan Pérez; Ugo I Ekeowa; Nedim Hadzic; Noor Kalsheker; Bibek Gooptu; Bernard Portmann; Didier Belorgey; Marian Hill; Susan Chambers; Jeff Teckman; Graeme J Alexander; Stefan J Marciniak; David A Lomas
Journal:  Hepatology       Date:  2010-09       Impact factor: 17.425

6.  Pathogenic alpha 1-antitrypsin polymers are formed by reactive loop-beta-sheet A linkage.

Authors:  P Sivasothy; T R Dafforn; P G Gettins; D A Lomas
Journal:  J Biol Chem       Date:  2000-10-27       Impact factor: 5.157

7.  Kinetic instability of the serpin Z alpha1-antitrypsin promotes aggregation.

Authors:  Anja S Knaupp; Vita Levina; Amy L Robertson; Mary C Pearce; Stephen P Bottomley
Journal:  J Mol Biol       Date:  2009-11-26       Impact factor: 5.469

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

Authors:  Masayuki Yamasaki; Wei Li; Daniel J D Johnson; James A Huntington
Journal:  Nature       Date:  2008-10-15       Impact factor: 49.962

  8 in total
  7 in total

1.  Conformational properties of the disease-causing Z variant of α1-antitrypsin revealed by theory and experiment.

Authors:  Itamar Kass; Anja S Knaupp; Stephen P Bottomley; Ashley M Buckle
Journal:  Biophys J       Date:  2012-06-19       Impact factor: 4.033

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Journal:  Endocr Rev       Date:  2014-03-24       Impact factor: 19.871

Review 3.  ELANE mutations in cyclic and severe congenital neutropenia: genetics and pathophysiology.

Authors:  Marshall S Horwitz; Seth J Corey; H Leighton Grimes; Timothy Tidwell
Journal:  Hematol Oncol Clin North Am       Date:  2012-11-07       Impact factor: 3.722

4.  A phenotypic high throughput screening assay for the identification of pharmacoperones for the gonadotropin releasing hormone receptor.

Authors:  P Michael Conn; Emery Smith; Timothy Spicer; Peter Chase; Louis Scampavia; Jo Ann Janovick
Journal:  Assay Drug Dev Technol       Date:  2014-05       Impact factor: 1.738

Review 5.  Transitioning pharmacoperones to therapeutic use: in vivo proof-of-principle and design of high throughput screens.

Authors:  P Michael Conn; David C Smithson; Peter S Hodder; M David Stewart; Richard R Behringer; Emery Smith; Alfredo Ulloa-Aguirre; Jo Ann Janovick
Journal:  Pharmacol Res       Date:  2013-12-25       Impact factor: 7.658

6.  Reglucosylation by UDP-glucose:glycoprotein glucosyltransferase 1 delays glycoprotein secretion but not degradation.

Authors:  Abla Tannous; Nishant Patel; Taku Tamura; Daniel N Hebert
Journal:  Mol Biol Cell       Date:  2014-11-26       Impact factor: 4.138

7.  A novel SERPINA1 mutation causing serum alpha(1)-antitrypsin deficiency.

Authors:  Darren N Saunders; Elizabeth A Tindall; Robert F Shearer; Jacquelyn Roberson; Amy Decker; Jean Amos Wilson; Vanessa M Hayes
Journal:  PLoS One       Date:  2012-12-12       Impact factor: 3.240

  7 in total

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