Pharmacoproteomics pinpoints HSP70 interaction for correction of the most frequent Wilson disease-causing mutant of ATP7B

Characterization of the most frequent ATP7B mutation causing Wilson disease in hepatocytes from patient induced pluripotent stem cells

(2018) Silvia Parisi et al.   Scientific Reports

Pathways of cellular proteostasis in aging and disease

(2017) Courtney L. Klaips et al.   JOURNAL OF CELL BIOLOGY

Identification of p38 MAPK and JNK as new targets for correction of Wilson disease-causing ATP7B mutants

(2016) Giancarlo Chesi et al.   HEPATOLOGY

A systems biology approach reveals new endoplasmic reticulum-associated targets for the correction of the ATP7B mutant causing Wilson disease

(2016) Mafalda Concilli et al.   Metallomics

Functional analysis and drug response to zinc and D-penicillamine in stableATP7Bmutant hepatic cell lines

(2016) Gursimran Chandhok et al.   WORLD JOURNAL OF GASTROENTEROLOGY

Geographic distribution of ATP7B mutations in Wilson disease

(2015) Amanda Gomes et al.   ANNALS OF HUMAN BIOLOGY

Quantitative Proteomics of Human Fibroblasts with I1061T Mutation in Niemann–Pick C1 (NPC1) Protein Provides Insights into the Disease Pathogenesis

(2015) Navin Rauniyar et al.   MOLECULAR & CELLULAR PROTEOMICS

∆F508 CFTR interactome remodelling promotes rescue of cystic fibrosis

(2015) Sandra Pankow et al.   NATURE

Identification of an Allosteric Small-Molecule Inhibitor Selective for the Inducible Form of Heat Shock Protein 70

(2014) Matthew K. Howe et al.   CHEMISTRY & BIOLOGY

Wilson Disease Protein ATP7B Utilizes Lysosomal Exocytosis to Maintain Copper Homeostasis

(2014) Elena V. Polishchuk et al.   DEVELOPMENTAL CELL

The role of the cytosolic HSP70 chaperone system in diseases caused by misfolding and aberrant trafficking of ion channels

(2014) J. C. Young   Disease Models & Mechanisms

ER-associated degradation: Protein quality control and beyond

(2014) Annamaria Ruggiano et al.   JOURNAL OF CELL BIOLOGY

SLC30A10 Is a Cell Surface-Localized Manganese Efflux Transporter, and Parkinsonism-Causing Mutations Block Its Intracellular Trafficking and Efflux Activity

(2014) D. Leyva-Illades et al.   JOURNAL OF NEUROSCIENCE

The Effect of Zinc and D-Penicillamine in a Stable Human Hepatoma ATP7B Knockout Cell Line

(2014) Gursimran Chandhok et al.   PLoS One

The cytosolic chaperone  -crystallin B rescues folding and compartmentalization of misfolded multispan transmembrane proteins

(2013) M. D'Agostino et al.   JOURNAL OF CELL SCIENCE

Diverse Functional Properties of Wilson Disease ATP7B Variants

(2012) Dominik Huster et al.   GASTROENTEROLOGY

Rescue of the mutant CFTR chloride channel by pharmacological correctors and low temperature analyzed by gene expression profiling

(2011) Elvira Sondo et al.   AMERICAN JOURNAL OF PHYSIOLOGY-CELL PHYSIOLOGY

Clusterin (Apolipoprotein J), a Molecular Chaperone That Facilitates Degradation of the Copper-ATPases ATP7A and ATP7B

(2011) Stephanie Materia et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Correction of the F508del-CFTR protein processing defect in vitro by the investigational drug VX-809

(2011) F. Van Goor et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Influence of cell background on pharmacological rescue of mutant CFTR

(2010) Nicoletta Pedemonte et al.   AMERICAN JOURNAL OF PHYSIOLOGY-CELL PHYSIOLOGY

Modeling general proteostasis: proteome balance in health and disease

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

The endoplasmic reticulum–associated Hsp40 DNAJB12 and Hsc70 cooperate to facilitate RMA1 E3–dependent degradation of nascent CFTRΔF508

(2010) Diane E. Grove et al.   MOLECULAR BIOLOGY OF THE CELL

The HSP70 chaperone machinery: J proteins as drivers of functional specificity

(2010) Harm H. Kampinga et al.   NATURE REVIEWS MOLECULAR CELL BIOLOGY

The proteostasis boundary in misfolding diseases of membrane traffic

(2009) Darren M. Hutt et al.   FEBS LETTERS

Reduced expression of ATP7B affected by Wilson disease-causing mutations is rescued by pharmacological folding chaperones 4-phenylbutyrate and curcumin

(2009) Peter V. E. van den Berghe et al.   HEPATOLOGY

Diagnosis and treatment of Wilson disease: An update

(2008) Eve A. Roberts et al.   HEPATOLOGY

Cysteine String Protein Promotes Proteasomal Degradation of the Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) by Increasing Its Interaction with the C Terminus of Hsp70-interacting Protein and Promoting CFTR Ubiquitylation

(2008) Béla Z. Schmidt et al.   JOURNAL OF BIOLOGICAL CHEMISTRY