Histone Deacetylase Inhibitor (HDACi) Suberoylanilide Hydroxamic Acid (SAHA)-mediated Correction of α1-Antitrypsin Deficiency

Proteostasis, an Emerging Therapeutic Paradigm for Managing Inflammatory Airway Stress Disease

(2012) M. Bouchecareilh et al.   CURRENT MOLECULAR MEDICINE

Class I histone deacetylases localize to the endoplasmic reticulum and modulate the unfolded protein response

(2012) Soumen Kahali et al.   FASEB JOURNAL

Preventing and reversing the cellular consequences of Z alpha-1 antitrypsin accumulation by targeting s4A

(2012) Sam Alam et al.   JOURNAL OF HEPATOLOGY

Function and Molecular Mechanism of Acetylation in Autophagy Regulation

(2012) C. Yi et al.   SCIENCE

A Review of α1-Antitrypsin Deficiency

(2011) James K. Stoller et al.   AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE

Trichostatin A Abrogates Airway Constriction, but Not Inflammation, in Murine and Human Asthma Models

(2011) Audreesh Banerjee et al.   AMERICAN JOURNAL OF RESPIRATORY CELL AND MOLECULAR BIOLOGY

Decreased histone deacetylase 2 impairs Nrf2 activation by oxidative stress

(2011) Nicolas Mercado et al.   BIOCHEMICAL AND BIOPHYSICAL RESEARCH COMMUNICATIONS

The diverse members of the mammalian HSP70 machine show distinct chaperone-like activities

(2011) Jurre Hageman et al.   BIOCHEMICAL JOURNAL

Post-translational modifications of Hsp90 and their contributions to chaperone regulation

(2011) Mehdi Mollapour et al.   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR CELL RESEARCH

Defect of Adaptation to Hypoxia in Patients With COPD Due to Reduction of Histone Deacetylase 7

(2011) Masako To et al.   CHEST

Molecular basis of α1-antitrypsin deficiency revealed by the structure of a domain-swapped trimer

(2011) Masayuki Yamasaki et al.   EMBO REPORTS

α1-antitrypsin deficiency and inflammation

(2011) Ugo I Ekeowa et al.   Expert Review of Clinical Immunology

Alpha-1-Antitrypsin Deficiency in Early Childhood

(2011) Aleksandra Topic et al.   Fetal and Pediatric Pathology

Gene therapy for alpha-1 antitrypsin deficiency

(2011) T. R. Flotte et al.   HUMAN MOLECULAR GENETICS

Histone deacetylase inhibitors in the generation of the anti-tumour immune response

(2011) Graham R Leggatt et al.   IMMUNOLOGY AND CELL BIOLOGY

Control of NF-κB and Inflammation by the Unfolded Protein Response

(2011) Masanori Kitamura   INTERNATIONAL REVIEWS OF IMMUNOLOGY

Critical role of proteostasis-imbalance in pathogenesis of COPD and severe emphysema

(2011) Taehong Min et al.   JOURNAL OF MOLECULAR MEDICINE-JMM

Golgi localization of ERManI defines spatial separation of the mammalian glycoprotein quality control system

(2011) Shujuan Pan et al.   MOLECULAR BIOLOGY OF THE CELL

A Diversity of Assembly Mechanisms of a Generic Amyloid Fold

(2011) Timo Eichner et al.   MOLECULAR CELL

Unknown

(2011) Dan Christensen et al.   MOLECULAR MEDICINE

HDAC and HDACi: pathogenetic and mechanistic insights

(2011) Jenny Buckland   Nature Reviews Rheumatology

Malectin Participates in a Backup Glycoprotein Quality Control Pathway in the Mammalian ER

(2011) Carmela Galli et al.   PLoS One

SAHA Decreases HDAC 2 and 4 Levels In Vivo and Improves Molecular Phenotypes in the R6/2 Mouse Model of Huntington's Disease

(2011) Michal Mielcarek et al.   PLoS One

Histone deacetylase inhibitors prevent the degradation and restore the activity of glucocerebrosidase in Gaucher disease

(2011) J. Lu et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Histone deacetylase inhibitor treatment dramatically reduces cholesterol accumulation in Niemann-Pick type C1 mutant human fibroblasts

(2011) N. H. Pipalia et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Patterns of Inflammatory Responses in Large and Small Airways in Smokers with and without Chronic Obstructive Pulmonary Disease

(2011) Sergejs Isajevs et al.   RESPIRATION

The Unfolded Protein Response: From Stress Pathway to Homeostatic Regulation

(2011) P. Walter et al.   SCIENCE

Histone deacetylases as regulators of inflammation and immunity

(2011) Melanie R. Shakespear et al.   TRENDS IN IMMUNOLOGY

The Stress of Protein Misfolding: From Single Cells to Multicellular Organisms

(2011) T. Gidalevitz et al.   Cold Spring Harbor Perspectives in Biology

Hepatic Fibrosis and Carcinogenesis in  1-Antitrypsin Deficiency: A Prototype for Chronic Tissue Damage in Gain-of-Function Disorders

(2011) D. H. Perlmutter et al.   Cold Spring Harbor Perspectives in Biology

Chemical and Biological Approaches for Adapting Proteostasis to Ameliorate Protein Misfolding and Aggregation Diseases-Progress and Prognosis

(2011) S. L. Lindquist et al.   Cold Spring Harbor Perspectives in Biology

Aging as an Event of Proteostasis Collapse

(2011) R. C. Taylor et al.   Cold Spring Harbor Perspectives in Biology

Potential Agents for Treating Cystic Fibrosis: Cyclic Tetrapeptides That Restore Trafficking and Activity of ΔF508-CFTR

(2011) Darren M. Hutt et al.   ACS Medicinal Chemistry Letters

Inhibition of histone deacetylase causes emphysema

(2010) Shiro Mizuno et al.   AMERICAN JOURNAL OF PHYSIOLOGY-LUNG CELLULAR AND MOLECULAR PHYSIOLOGY

Molecular mechanisms of oxidative stress in airways and lungs with reference to asthma and chronic obstructive pulmonary disease

(2010) K.F. Chung et al.   Annals of the New York Academy of Sciences

Alpha-1-Antitrypsin Deficiency: Importance of Proteasomal and Autophagic Degradative Pathways in Disposal of Liver Disease–Associated Protein Aggregates

(2010) David H. Perlmutter   Annual Review of Medicine

Predicting post-translational lysine acetylation using support vector machines

(2010) F. Gnad et al.   BIOINFORMATICS

Chemical and/or biological therapeutic strategies to ameliorate protein misfolding diseases

(2010) Derrick Sek Tong Ong et al.   CURRENT OPINION IN CELL BIOLOGY

Modeling general proteostasis: proteome balance in health and disease

(2010) Daniela M Roth et al.   CURRENT OPINION IN CELL BIOLOGY

Overcoming Reduced Glucocorticoid Sensitivity in Airway Disease

(2010) John A. Marwick et al.   DRUGS

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

(2010) Shalesh Kaushal et al.   EXPERIMENTAL BIOLOGY AND MEDICINE

The clinical development of histone deacetylase inhibitors as targeted anticancer drugs

(2010) Paul A Marks   EXPERT OPINION ON INVESTIGATIONAL DRUGS

Nrf2-ARE stress response mechanism: A control point in oxidative stress-mediated dysfunctions and chronic inflammatory diseases

(2010) Shruti Singh et al.   FREE RADICAL RESEARCH

Physical and Functional HAT/HDAC Interplay Regulates Protein Acetylation Balance

(2010) Alessia Peserico et al.   JOURNAL OF BIOMEDICINE AND BIOTECHNOLOGY

Endoplasmic reticulum stress in liver disease

(2010) Harmeet Malhi et al.   JOURNAL OF HEPATOLOGY

Combined proteasome and histone deacetylase inhibition: A promising synergy for patients with relapsed/refractory multiple myeloma

(2010) Sundar Jagannath et al.   LEUKEMIA RESEARCH

Characterisation of serpin polymers in vitro and in vivo

(2010) Didier Belorgey et al.   METHODS

Autophagy and the Integrated Stress Response

(2010) Guido Kroemer et al.   MOLECULAR CELL

Heat shock factors: integrators of cell stress, development and lifespan

(2010) Malin Åkerfelt et al.   NATURE REVIEWS MOLECULAR CELL BIOLOGY

Nicotinamide improves motor deficits and upregulates PGC-1α and BDNF gene expression in a mouse model of Huntington's disease

(2010) Tyisha Hathorn et al.   NEUROBIOLOGY OF DISEASE

Alpha1-Antitrypsin Deficiency and Autophagy

(2010) Stefan J. Marciniak et al.   NEW ENGLAND JOURNAL OF MEDICINE

Automated High-Content Live Animal Drug Screening Using C. elegans Expressing the Aggregation Prone Serpin α1-antitrypsin Z

(2010) Sager J. Gosai et al.   PLoS One

Defining the mechanism of polymerization in the serpinopathies

(2010) U. I. Ekeowa et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Alpha-1 antitrypsin deficiency

(2010) Emer Kelly et al.   RESPIRATORY MEDICINE

The Proteome in Balance

(2010) D. Hutt et al.   SCIENCE

Clearing Conformational Disease

(2010) R. N. Sifers   SCIENCE

An Autophagy-Enhancing Drug Promotes Degradation of Mutant  1-Antitrypsin Z and Reduces Hepatic Fibrosis

(2010) T. Hidvegi et al.   SCIENCE

Protein quality control in the ER: The recognition of misfolded proteins

(2010) Pekka Määttänen et al.   SEMINARS IN CELL & DEVELOPMENTAL BIOLOGY

Role of the Lectin VIP36 in Post-ER Quality Control of Human α1-Antitrypsin

(2010) Veronika Reiterer et al.   TRAFFIC

Emergent Properties of Proteostasis in Managing Cystic Fibrosis

(2010) W. E. Balch et al.   Cold Spring Harbor Perspectives in Biology

Biological and Chemical Approaches to Diseases of Proteostasis Deficiency

(2009) Evan T. Powers et al.   Annual Review of Biochemistry

Conformational Pathology of the Serpins: Themes, Variations, and Therapeutic Strategies

(2009) Bibek Gooptu et al.   Annual Review of Biochemistry

Current Perspectives on Role of Chromatin Modifications and Deacetylases in Lung Inflammation in COPD

(2009) Saravanan Rajendrasozhan et al.   COPD-Journal of Chronic Obstructive Pulmonary Disease

Histone Deacetylase Inhibitors In Inflammatory Disease

(2009) Maria Halili et al.   CURRENT TOPICS IN MEDICINAL CHEMISTRY

Mechanisms of emphysema in  1-antitrypsin deficiency: molecular and cellular insights

(2009) B. Gooptu et al.   EUROPEAN RESPIRATORY JOURNAL

Silent information regulator, Sirtuin 1, and age-related diseases

(2009) Li Zeng et al.   Geriatrics & Gerontology International

Single nucleotide polymorphism-mediated translational suppression of endoplasmic reticulum mannosidase I modifies the onset of end-stage liver disease in alpha1-antitrypsin deficiency

(2009) Shujuan Pan et al.   HEPATOLOGY

Calnexin Phosphorylation Attenuates the Release of Partially Misfolded α1-Antitrypsin to the Secretory Pathway

(2009) Pamela H. Cameron et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

HDAC6 Modulates Hsp90 Chaperone Activity and Regulates Activation of Aryl Hydrocarbon Receptor Signaling

(2009) Vikram D. Kekatpure et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

EDEM1 Recognition and Delivery of Misfolded Proteins to the SEL1L-Containing ERAD Complex

(2009) James H. Cormier et al.   MOLECULAR CELL

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

(2009) Darren M Hutt et al.   Nature Chemical Biology

Stress-Inducible Regulation of Heat Shock Factor 1 by the Deacetylase SIRT1

(2009) S. D. Westerheide et al.   SCIENCE

Lysine Acetylation Targets Protein Complexes and Co-Regulates Major Cellular Functions

(2009) C. Choudhary et al.   SCIENCE

Multiple roles of HDAC inhibition in neurodegenerative conditions

(2009) De-Maw Chuang et al.   TRENDS IN NEUROSCIENCES

Proteotoxic stress and inducible chaperone networks in neurodegenerative disease and aging

(2008) R. I. Morimoto   GENES & DEVELOPMENT

A Chicken Ovalbumin Upstream Promoter Transcription Factor I (COUP-TFI) Complex Represses Expression of the Gene Encoding Tumor Necrosis Factor α-induced Protein 8 (TNFAIP8)

(2008) Ling-juan Zhang et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Identification of ERGIC-53 as an intracellular transport receptor of α1-antitrypsin

(2008) Beat Nyfeler et al.   JOURNAL OF CELL BIOLOGY

OS-9 and GRP94 deliver mutant α1-antitrypsin to the Hrd1–SEL1L ubiquitin ligase complex for ERAD

(2008) John C. Christianson et al.   NATURE CELL BIOLOGY

The HDAC inhibitor 4b ameliorates the disease phenotype and transcriptional abnormalities in Huntington's disease transgenic mice

(2008) E. A. Thomas et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Adapting Proteostasis for Disease Intervention

(2008) William E. Balch et al.   SCIENCE