The mammalian unfolded protein response
M Schröder, RJ Kaufman - Annu. Rev. Biochem., 2005 - annualreviews.org
▪ Abstract In the endoplasmic reticulum (ER), secretory and transmembrane proteins fold into
their native conformation and undergo posttranslational modifications important for their …
their native conformation and undergo posttranslational modifications important for their …
CFTR function and prospects for therapy
JR Riordan - Annu. Rev. Biochem., 2008 - annualreviews.org
Mutations in the gene coding for the cystic fibrosis transmembrane conductance regulator
(CFTR) epithelial anion channel cause cystic fibrosis (CF). The multidomain integral …
(CFTR) epithelial anion channel cause cystic fibrosis (CF). The multidomain integral …
Ca2+ store depletion causes STIM1 to accumulate in ER regions closely associated with the plasma membrane
MM Wu, JA Buchanan, RM Luik, RS Lewis - The Journal of cell biology, 2006 - rupress.org
Stromal interacting molecule 1 (STIM1), reported to be an endoplasmic reticulum (ER) Ca2+
sensor controlling store-operated Ca2+ entry, redistributes from a diffuse ER localization into …
sensor controlling store-operated Ca2+ entry, redistributes from a diffuse ER localization into …
Curcumin, a major constituent of turmeric, corrects cystic fibrosis defects
ME Egan, M Pearson, SA Weiner, V Rajendran… - Science, 2004 - science.org
Cystic fibrosis is caused by mutations in the gene encoding the cystic fibrosis
transmembrane conductance regulator (CFTR). The most common mutation, ΔF508, results …
transmembrane conductance regulator (CFTR). The most common mutation, ΔF508, results …
[HTML][HTML] Rescue of ΔF508-CFTR trafficking via a GRASP-dependent unconventional secretion pathway
The most prevalent disease-causing mutation of CFTR is the deletion of Phe508 (ΔF508),
which leads to defects in conventional Golgi-mediated exocytosis and cell surface …
which leads to defects in conventional Golgi-mediated exocytosis and cell surface …
The intrinsic and extrinsic effects of N-linked glycans on glycoproteostasis
Proteins that traffic through the eukaryotic secretory pathway are commonly modified with N-
linked carbohydrates. These bulky amphipathic modifications at asparagines intrinsically …
linked carbohydrates. These bulky amphipathic modifications at asparagines intrinsically …
[HTML][HTML] NPHS2 mutation analysis shows genetic heterogeneityof steroid-resistant nephrotic syndrome and lowpost-transplant recurrence
S Weber, O Gribouval, EL Esquivel, V Morinière… - Kidney international, 2004 - Elsevier
NPHS2 mutation analysis shows genetic heterogeneity of steroid-resistant nephrotic
syndrome and low post-transplant recurrence. Background Mutations of NPHS2 are …
syndrome and low post-transplant recurrence. Background Mutations of NPHS2 are …
Curcumin decreases amyloid-β peptide levels by attenuating the maturation of amyloid-β precursor protein
Alzheimer disease (AD) is a devastating neurodegenerative disease with no cure. The
pathogenesis of AD is believed to be driven primarily by amyloid-β (Aβ), the principal …
pathogenesis of AD is believed to be driven primarily by amyloid-β (Aβ), the principal …
Endoplasmic reticulum–associated degradation
K Römisch - Annu. Rev. Cell Dev. Biol., 2005 - annualreviews.org
Secretory and transmembrane proteins enter the secretory pathway through the protein-
conducting Sec61 channel in the membrane of the endoplasmic reticulum. In the …
conducting Sec61 channel in the membrane of the endoplasmic reticulum. In the …
Nanoparticles that deliver triplex-forming peptide nucleic acid molecules correct F508del CFTR in airway epithelium
NA McNeer, K Anandalingam, RJ Fields… - Nature …, 2015 - nature.com
Cystic fibrosis (CF) is a lethal genetic disorder most commonly caused by the F508del
mutation in the cystic fibrosis transmembrane conductance regulator (CFTR) gene. It is not …
mutation in the cystic fibrosis transmembrane conductance regulator (CFTR) gene. It is not …