Carbamazepine as a Novel Small Molecule Corrector of Trafficking-impaired ATP-sensitive Potassium Channels Identified in Congenital Hyperinsulinism

Compounds that correct F508del-CFTR trafficking can also correct other protein trafficking diseases: an in vitro study using cell lines

(2013) Heidi M Sampson et al.   Orphanet Journal of Rare Diseases

Role of Derlin-1 Protein in Proteostasis Regulation of ATP-sensitive Potassium Channels

(2012) Fang Wang et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Genotype and Phenotype Correlations in 417 Children With Congenital Hyperinsulinism

(2012) K. E. Snider et al.   JOURNAL OF CLINICAL ENDOCRINOLOGY & METABOLISM

BAD-Dependent Regulation of Fuel Metabolism and KATP Channel Activity Confers Resistance to Epileptic Seizures

(2012) Alfredo Giménez-Cassina et al.   NEURON

Functional Rescue of F508del-CFTR Using Small Molecule Correctors

(2012) Steven Molinski et al.   Frontiers in Pharmacology

Syntaxin 1A regulates surface expression of β-cell ATP-sensitive potassium channels

(2011) Pei-Chun Chen et al.   AMERICAN JOURNAL OF PHYSIOLOGY-CELL PHYSIOLOGY

Identification of a NBD1-Binding Pharmacological Chaperone that Corrects the Trafficking Defect of F508del-CFTR

(2011) Heidi M. Sampson et al.   CHEMISTRY & BIOLOGY

Diazoxide-Unresponsive Congenital Hyperinsulinism in Children With Dominant Mutations of the  -Cell Sulfonylurea Receptor SUR1

(2011) C. M. MacMullen et al.   DIABETES

CFTR: folding, misfolding and correcting the ΔF508 conformational defect

(2011) Gergely L. Lukacs et al.   TRENDS IN MOLECULAR MEDICINE

Chemical Inducers of Autophagy That Enhance the Clearance of Mutant Proteins in Neurodegenerative Diseases

(2010) Maurizio Renna et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Role of Hsp90 in Biogenesis of the β-Cell ATP-sensitive Potassium Channel Complex

(2010) Fei-Fei Yan et al.   MOLECULAR BIOLOGY OF THE CELL

Correction of the  Phe508 Cystic Fibrosis Transmembrane Conductance Regulator Trafficking Defect by the Bioavailable Compound Glafenine

(2010) R. Robert et al.   MOLECULAR PHARMACOLOGY

Defective CFTR induces aggresome formation and lung inflammation in cystic fibrosis through ROS-mediated autophagy inhibition

(2010) Alessandro Luciani et al.   NATURE CELL BIOLOGY

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

Biological and Chemical Approaches to Diseases of Proteostasis Deficiency

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

Destabilization of ATP-sensitive Potassium Channel Activity by NovelKCNJ11Mutations Identified in Congenital Hyperinsulinism

(2008) Yu-Wen Lin et al.   JOURNAL OF BIOLOGICAL CHEMISTRY