Endoplasmic reticulum stress as a therapeutic target in cardiovascular disease

T Minamino, I Komuro, M Kitakaze - Circulation research, 2010 - Am Heart Assoc
Cardiovascular disease constitutes a major and increasing health burden in developed
countries. Although treatments have progressed, the development of novel treatments for …

The delicate balance between secreted protein folding and endoplasmic reticulum-associated degradation in human physiology

CJ Guerriero, JL Brodsky - Physiological reviews, 2012 - journals.physiology.org
Protein folding is a complex, error-prone process that often results in an irreparable protein
by-product. These by-products can be recognized by cellular quality control machineries …

Rescue of ΔF508-CFTR trafficking and gating in human cystic fibrosis airway primary cultures by small molecules

F Van Goor, KS Straley, D Cao… - … of Physiology-Lung …, 2006 - journals.physiology.org
Cystic fibrosis (CF) is a fatal genetic disease caused by mutations in cftr, a gene encoding a
PKA-regulated Cl− channel. The most common mutation results in a deletion of …

Hsp90 cochaperone Aha1 downregulation rescues misfolding of CFTR in cystic fibrosis

X Wang, J Venable, P LaPointe, DM Hutt, AV Koulov… - Cell, 2006 - cell.com
The pathways that distinguish transport of folded and misfolded cargo through the exocytic
(secretory) pathway of eukaryotic cells remain unknown. Using proteomics to assess global …

Genetic diseases of connective tissues: cellular and extracellular effects of ECM mutations

JF Bateman, RP Boot-Handford… - Nature Reviews …, 2009 - nature.com
Tissue-specific extracellular matrices (ECMs) are crucial for normal development and tissue
function, and mutations in ECM genes result in a wide range of serious inherited connective …

[HTML][HTML] Chemical and biological approaches synergize to ameliorate protein-folding diseases

TW Mu, DST Ong, YJ Wang, WE Balch, JR Yates… - Cell, 2008 - cell.com
Loss-of-function diseases are often caused by a mutation in a protein traversing the
secretory pathway that compromises the normal balance between protein folding, trafficking …

N-glycan structure dictates extension of protein folding or onset of disposal

M Molinari - Nature chemical biology, 2007 - nature.com
The endoplasmic reticulum (ER) is the site of folding for proteins that are resident in the ER
or that are destined for the Golgi, endosomes, lysosomes, the plasma membrane, or …

Expanding the number of 'druggable'targets: non‐enzymes and protein–protein interactions

LN Makley, JE Gestwicki - Chemical biology & drug design, 2013 - Wiley Online Library
Following sequencing and assembly of the human genome, the preferred methods for
identification of new drug targets have changed dramatically. Modern tactics such as …

[HTML][HTML] Regulation of G protein-coupled receptor export trafficking

C Dong, CM Filipeanu, MT Duvernay, G Wu - Biochimica et Biophysica Acta …, 2007 - Elsevier
G protein-coupled receptors (GPCRs) constitute a superfamily of cell-surface receptors
which share a common topology of seven transmembrane domains and modulate a variety …

Molecular biology of hereditary diabetes insipidus

TM Fujiwara, DG Bichet - Journal of the American Society of …, 2005 - journals.lww.com
The identification, characterization, and mutational analysis of three different genes—the
arginine vasopressin gene (AVP), the arginine vasopressin receptor 2 gene (AVPR2), and …