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Diversity in the origins of proteostasis networks--a driver for protein function in evolution

Nature reviews. Molecular cell biology, 2013-04, Vol.14 (4), p.237-248

2013

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Adapting Proteostasis for Disease Intervention

Science (American Association for the Advancement of Science), 2008-02, Vol.319 (5865), p.916-919

2008

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Biological and chemical approaches to diseases of proteostasis deficiency

Annual review of biochemistry, 2009-01, Vol.78 (1), p.959-991

2009

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Functional amyloid--from bacteria to humans

Trends in biochemical sciences (Amsterdam. Regular ed.), 2007-05, Vol.32 (5), p.217-224

2007

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From CFTR biology toward combinatorial pharmacotherapy: expanded classification of cystic fibrosis mutations

Molecular biology of the cell, 2016-02, Vol.27 (3), p.424-433

2016

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Tracing genetic diversity captures the molecular basis of misfolding disease

Nature communications, 2024-04, Vol.15 (1), p.3333-3333, Article 3333

2024

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COPII cage assembly factor Sec13 integrates information flow regulating endomembrane function in response to human variation

Scientific reports, 2024-05, Vol.14 (1), p.10160-10160, Article 10160

2024

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The Biological and Chemical Basis for Tissue-Selective Amyloid Disease

Cell, 2005-04, Vol.121 (1), p.73-85

2005

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Functional amyloid formation within mammalian tissue

PLoS biology, 2006-01, Vol.4 (1), p.e6

2006

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Modulation of the maladaptive stress response to manage diseases of protein folding

PLoS biology, 2014-11, Vol.12 (11), p.e1001998

2014

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A chaperone trap contributes to the onset of cystic fibrosis

PloS one, 2012-05, Vol.7 (5), p.e37682-e37682

2012

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Histone deacetylase inhibitors correct the cholesterol storage defect in most Niemann-Pick C1 mutant cells

Journal of lipid research, 2017-04, Vol.58 (4), p.695-708

2017

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A Di-Acidic Signal Required for Selective Export from the Endoplasmic Reticulum

Science (American Association for the Advancement of Science), 1997-07, Vol.277 (5325), p.556-558

1997

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Structural Basis for Cargo Regulation of COPII Coat Assembly

Cell, 2008-08, Vol.134 (3), p.474-484

2008

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Quantitating the epigenetic transformation contributing to cholesterol homeostasis using Gaussian process

Nature communications, 2019-11, Vol.10 (1), p.5052-15, Article 5052

2019

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Proteostasis, an emerging therapeutic paradigm for managing inflammatory airway stress disease

Current molecular medicine, 2012-08, Vol.12 (7), p.815-826

2012

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Hsp90 Cochaperone Aha1 Downregulation Rescues Misfolding of CFTR in Cystic Fibrosis

Cell, 2006-11, Vol.127 (4), p.803-815

2006

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Sterol O-Acyltransferase 1 ( SOAT1 ): A Genetic Modifier of Niemann-Pick Disease, Type C1

International journal of molecular sciences, 2024-04, Vol.25 (8), p.4217

2024

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NPC1/NPC2 function as a tag team duo to mobilize cholesterol

Proceedings of the National Academy of Sciences - PNAS, 2008-10, Vol.105 (40), p.15223-15224

2008

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Chemical Chaperones Increase the Cellular Activity of N370S β-Glucosidase: A Therapeutic Strategy for Gaucher Disease

Proceedings of the National Academy of Sciences - PNAS, 2002-11, Vol.99 (24), p.15428-15433

2002

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Reduced histone deacetylase 7 activity restores function to misfolded CFTR in cystic fibrosis

Nature chemical biology, 2010-01, Vol.6 (1), p.25-33

2010

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Quantitative Analysis of the Proteome Response to the Histone Deacetylase Inhibitor (HDACi) Vorinostat in Niemann-Pick Type C1 disease

Molecular & cellular proteomics, 2017-11, Vol.16 (11), p.1938-1957

2017

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Spatial covariance analysis reveals the residue-by-residue thermodynamic contribution of variation to the CFTR fold

Communications biology, 2022-04, Vol.5 (1), p.356-356, Article 356

2022

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Biological and structural basis for Aha1 regulation of Hsp90 ATPase activity in maintaining proteostasis in the human disease cystic fibrosis

Molecular biology of the cell, 2010-03, Vol.21 (6), p.871-884

2010

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Rab1 Recruitment of p115 into a cis-SNARE Complex: Programming Budding COPII Vesicles for Fusion

Science (American Association for the Advancement of Science), 2000-07, Vol.289 (5478), p.444-448

2000

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Diversity in the origins of proteostasis networks--a driver for protein function in evolution

Adapting Proteostasis for Disease Intervention

Biological and chemical approaches to diseases of proteostasis deficiency

Functional amyloid--from bacteria to humans

From CFTR biology toward combinatorial pharmacotherapy: expanded classification of cystic fibrosis mutations

Tracing genetic diversity captures the molecular basis of misfolding disease

COPII cage assembly factor Sec13 integrates information flow regulating endomembrane function in response to human variation

The Biological and Chemical Basis for Tissue-Selective Amyloid Disease

Functional amyloid formation within mammalian tissue

Modulation of the maladaptive stress response to manage diseases of protein folding

A chaperone trap contributes to the onset of cystic fibrosis

Histone deacetylase inhibitors correct the cholesterol storage defect in most Niemann-Pick C1 mutant cells

A Di-Acidic Signal Required for Selective Export from the Endoplasmic Reticulum

Structural Basis for Cargo Regulation of COPII Coat Assembly

Quantitating the epigenetic transformation contributing to cholesterol homeostasis using Gaussian process

Proteostasis, an emerging therapeutic paradigm for managing inflammatory airway stress disease

Hsp90 Cochaperone Aha1 Downregulation Rescues Misfolding of CFTR in Cystic Fibrosis

Sterol O-Acyltransferase 1 ( SOAT1 ): A Genetic Modifier of Niemann-Pick Disease, Type C1

NPC1/NPC2 function as a tag team duo to mobilize cholesterol

Chemical Chaperones Increase the Cellular Activity of N370S β-Glucosidase: A Therapeutic Strategy for Gaucher Disease

Reduced histone deacetylase 7 activity restores function to misfolded CFTR in cystic fibrosis

Quantitative Analysis of the Proteome Response to the Histone Deacetylase Inhibitor (HDACi) Vorinostat in Niemann-Pick Type C1 disease

Spatial covariance analysis reveals the residue-by-residue thermodynamic contribution of variation to the CFTR fold

Biological and structural basis for Aha1 regulation of Hsp90 ATPase activity in maintaining proteostasis in the human disease cystic fibrosis

Rab1 Recruitment of p115 into a cis-SNARE Complex: Programming Budding COPII Vesicles for Fusion

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