Literature DB >> 12417557

The cellular response to aggregated proteins associated with human disease.

David H Perlmutter1.   

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Year:  2002        PMID： 12417557      PMCID： PMC151619          DOI： 10.1172/JCI16780

Source DB:  PubMed          Journal:  J Clin Invest        ISSN： 0021-9738            Impact factor:   14.808


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  14 in total

Review 1.  The action of molecular chaperones in the early secretory pathway.

Authors:  S W Fewell; K J Travers; J S Weissman; J L Brodsky
Journal:  Annu Rev Genet       Date:  2001       Impact factor: 16.830

2.  Naturally secreted oligomers of amyloid beta protein potently inhibit hippocampal long-term potentiation in vivo.

Authors:  Dominic M Walsh; Igor Klyubin; Julia V Fadeeva; William K Cullen; Roger Anwyl; Michael S Wolfe; Michael J Rowan; Dennis J Selkoe
Journal:  Nature       Date:  2002-04-04       Impact factor: 49.962

Review 3.  Alpha1-antitrypsin polymerization and the serpinopathies: pathobiology and prospects for therapy.

Authors:  David A Lomas; Ravi Mahadeva
Journal:  J Clin Invest       Date:  2002-12       Impact factor: 14.808

Review 4.  Rescuing protein conformation: prospects for pharmacological therapy in cystic fibrosis.

Authors:  Marina S Gelman; Ron R Kopito
Journal:  J Clin Invest       Date:  2002-12       Impact factor: 14.808

Review 5.  Orchestrating the unfolded protein response in health and disease.

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

6.  Role of the proteasome in membrane extraction of a short-lived ER-transmembrane protein.

Authors:  T U Mayer; T Braun; S Jentsch
Journal:  EMBO J       Date:  1998-06-15       Impact factor: 11.598

7.  Principles that govern the folding of protein chains.

Authors:  C B Anfinsen
Journal:  Science       Date:  1973-07-20       Impact factor: 47.728

8.  Familial dementia caused by polymerization of mutant neuroserpin.

Authors:  R L Davis; A E Shrimpton; P D Holohan; C Bradshaw; D Feiglin; G H Collins; P Sonderegger; J Kinter; L M Becker; F Lacbawan; D Krasnewich; M Muenke; D A Lawrence; M S Yerby; C M Shaw; B Gooptu; P R Elliott; J T Finch; R W Carrell; D A Lomas
Journal:  Nature       Date:  1999-09-23       Impact factor: 49.962

9.  Association of calnexin with mutant peripheral myelin protein-22 ex vivo: a basis for "gain-of-function" ER diseases.

Authors:  K M Dickson; J J M Bergeron; I Shames; J Colby; D T Nguyen; E Chevet; D Y Thomas; G J Snipes
Journal:  Proc Natl Acad Sci U S A       Date:  2002-07-15       Impact factor: 11.205

10.  Inherent toxicity of aggregates implies a common mechanism for protein misfolding diseases.

Authors:  Monica Bucciantini; Elisa Giannoni; Fabrizio Chiti; Fabiana Baroni; Lucia Formigli; Jesús Zurdo; Niccolò Taddei; Giampietro Ramponi; Christopher M Dobson; Massimo Stefani
Journal:  Nature       Date:  2002-04-04       Impact factor: 49.962
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  10 in total

1.  Sequestration of mutated alpha1-antitrypsin into inclusion bodies is a cell-protective mechanism to maintain endoplasmic reticulum function.

Authors:  Susana Granell; Giovanna Baldini; Sameer Mohammad; Vanessa Nicolin; Paola Narducci; Brian Storrie; Giulia Baldini
Journal:  Mol Biol Cell       Date:  2007-11-28       Impact factor: 4.138

Review 2.  Carbonic anhydrase as a model for biophysical and physical-organic studies of proteins and protein-ligand binding.

Authors:  Vijay M Krishnamurthy; George K Kaufman; Adam R Urbach; Irina Gitlin; Katherine L Gudiksen; Douglas B Weibel; George M Whitesides
Journal:  Chem Rev       Date:  2008-03       Impact factor: 60.622

Review 3.  Mechanism and Regulation of Autophagy and Its Role in Neuronal Diseases.

Authors:  Zhiping Hu; Binbin Yang; Xiaoye Mo; Han Xiao
Journal:  Mol Neurobiol       Date:  2014-10-15       Impact factor: 5.590

4.  The aggregation-prone intracellular serpin SRP-2 fails to transit the ER in Caenorhabditis elegans.

Authors:  Richard M Silverman; Erin E Cummings; Linda P O'Reilly; Mark T Miedel; Gary A Silverman; Cliff J Luke; David H Perlmutter; Stephen C Pak
Journal:  Genetics       Date:  2015-03-18       Impact factor: 4.562

5.  Rapamycin reduces intrahepatic alpha-1-antitrypsin mutant Z protein polymers and liver injury in a mouse model.

Authors:  Shalesh Kaushal; Mani Annamali; Keith Blomenkamp; David Rudnick; Donna Halloran; Elizabeth M Brunt; Jeffrey H Teckman
Journal:  Exp Biol Med (Maywood)       Date:  2010-06

6.  Linking of autophagy to ubiquitin-proteasome system is important for the regulation of endoplasmic reticulum stress and cell viability.

Authors:  Wen-Xing Ding; Hong-Min Ni; Wentao Gao; Tamotsu Yoshimori; Donna B Stolz; David Ron; Xiao-Ming Yin
Journal:  Am J Pathol       Date:  2007-07-09       Impact factor: 4.307

7.  A genome-wide RNAi screen identifies potential drug targets in a C. elegans model of α1-antitrypsin deficiency.

Authors:  Linda P O'Reilly; Olivia S Long; Murat C Cobanoglu; Joshua A Benson; Cliff J Luke; Mark T Miedel; Pamela Hale; David H Perlmutter; Ivet Bahar; Gary A Silverman; Stephen C Pak
Journal:  Hum Mol Genet       Date:  2014-05-16       Impact factor: 6.150

8.  Deficient and Null Variants of SERPINA1 Are Proteotoxic in a Caenorhabditis elegans Model of α1-Antitrypsin Deficiency.

Authors:  Erin E Cummings; Linda P O'Reilly; Dale E King; Richard M Silverman; Mark T Miedel; Cliff J Luke; David H Perlmutter; Gary A Silverman; Stephen C Pak
Journal:  PLoS One       Date:  2015-10-29       Impact factor: 3.240

9.  Tauroursodeoxycholic bile acid arrests axonal degeneration by inhibiting the unfolded protein response in X-linked adrenoleukodystrophy.

Authors:  Nathalie Launay; Montserrat Ruiz; Laia Grau; Francisco J Ortega; Ekaterina V Ilieva; Juan José Martínez; Elena Galea; Isidre Ferrer; Erwin Knecht; Aurora Pujol; Stéphane Fourcade
Journal:  Acta Neuropathol       Date:  2016-12-21       Impact factor: 17.088

10.  CemOrange2 fusions facilitate multifluorophore subcellular imaging in C. elegans.

Authors:  Brian J Thomas; Ira E Wight; Wendy Y Y Chou; Marco Moreno; Zachary Dawson; Arielle Homayouni; Huiyan Huang; Hyori Kim; Hanna Jia; Justin R Buland; Jennifer A Wambach; F Sessions Cole; Stephen C Pak; Gary A Silverman; Cliff J Luke
Journal:  PLoS One       Date:  2019-03-26       Impact factor: 3.240
  10 in total

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