Remodeling the Proteostasis Network to Rescue Glucocerebrosidase Variants by Inhibiting ER-Associated Degradation and Enhancing ER Folding

Review of miglustat for clinical management in Gaucher disease type 1

(2016) Can Ficicioglu   Therapeutics and Clinical Risk Management

Lacidipine Remodels Protein Folding and Ca2+ Homeostasis in Gaucher's Disease Fibroblasts: A Mechanism to Rescue Mutant Glucocerebrosidase

(2011) Fan Wang et al.   CHEMISTRY & BIOLOGY

Inhibition of Endoplasmic Reticulum-associated Degradation Rescues Native Folding in Loss of Function Protein Misfolding Diseases

(2011) Fan Wang et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

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

Ca2+Homeostasis Modulation Enhances the Amenability of L444P Glucosylcerebrosidase to Proteostasis Regulation in Patient-Derived Fibroblasts

(2010) Fan Wang et al.   ACS Chemical Biology

The pharmacological chaperone isofagomine increases the activity of the Gaucher disease L444P mutant form of β-glucosidase

(2010) Richie Khanna et al.   FEBS Journal

Apoptosis Protection by Mcl-1 and Bcl-2 Modulation of Inositol 1,4,5-Trisphosphate Receptor-dependent Ca2+Signaling

(2010) Emily F. Eckenrode et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

The BCL-2 Family Reunion

(2010) Jerry E. Chipuk et al.   MOLECULAR CELL

Endoplasmic reticulum Ca2+ increases enhance mutant glucocerebrosidase proteostasis

(2010) Derrick Sek Tong Ong et al.   Nature Chemical Biology

The ERAD Inhibitor Eeyarestatin I Is a Bifunctional Compound with a Membrane-Binding Domain and a p97/VCP Inhibitory Group

(2010) Qiuyan Wang et al.   PLoS One

HSP72 Protects Cells from ER Stress-induced Apoptosis via Enhancement of IRE1α-XBP1 Signaling through a Physical Interaction

(2010) Sanjeev Gupta et al.   PLOS BIOLOGY

Divergent Effects of PERK and IRE1 Signaling on Cell Viability

(2009) Jonathan H. Lin et al.   PLoS One

The BH4 domain of Bcl-2 inhibits ER calcium release and apoptosis by binding the regulatory and coupling domain of the IP3 receptor

(2009) Y.-P. Rong et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Chemical and Biological Approaches Synergize to Ameliorate Protein-Folding Diseases

(2008) Ting-Wei Mu et al.   CELL

Gaucher disease: mutation and polymorphism spectrum in the glucocerebrosidase gene (GBA)

(2008) Kathleen S. Hruska et al.   HUMAN MUTATION

Fluvastatin reduces oxidative damage in human vascular endothelial cells by upregulating Bcl-2

(2008) S. Z. XU et al.   JOURNAL OF THROMBOSIS AND HAEMOSTASIS

The BCL-2 protein family: opposing activities that mediate cell death

(2007) Richard J. Youle et al.   NATURE REVIEWS MOLECULAR CELL BIOLOGY