The endoplasmic reticulum (ER) functions as a quality-control organelle for protein
homeostasis, or “proteostasis”. The protein quality control systems involve ER-associated …

The clinical manifestation of neurodegenerative diseases is initiated by the selective
alteration in the functionality of distinct neuronal populations. The pathology of many …

Retinitis Pigmentosa (RP) is a hereditary retinopathy that affects about 2.5 million people
worldwide. It is characterized with progressive loss of rods and cones and causes severe …

Retinitis pigmentosa (RP) is the most common cause of inherited blindness and is
characterised by the progressive loss of retinal photoreceptors. However, RP is a highly …

Inherited mutations in the rod visual pigment, rhodopsin, cause the degenerative blinding
condition, retinitis pigmentosa (RP). Over 150 different mutations in rhodopsin have been …

Stress induced by the accumulation of unfolded proteins in the endoplasmic reticulum (ER)
is a feature of specialized secretory cells and is also observed in many diseases, including …

Protein homeostasis (proteostasis) requires the timely degradation of misfolded proteins and
their aggregates by protein quality control (PQC), of which molecular chaperones are an …

The unfolded protein response (UPR) is a homeostatic mechanism by which cells regulate
levels of misfolded proteins in the endoplasmic reticulum (ER). Although it is well …

Advances over the past 25 years have revealed much about how the structural properties of
membranes and associated proteins are linked to the thermodynamics and kinetics of …

Depending on endoplasmic reticulum (ER) stress levels, the ER transmembrane
multidomain protein IRE1α promotes either adaptation or apoptosis. Unfolded ER proteins …