The 70-kDa heat shock proteins (Hsp70s) are ubiquitous molecular chaperones that act in a
large variety of cellular protein folding and remodelling processes. They function virtually at …

Each protein must be synthesized with the correct amino acid sequence, folded into its
native structure, and transported to a relevant subcellular location and protein complex. If …

The ubiquitous heat shock protein 70 (HSP70) family consists of ATP-dependent molecular
chaperones, which perform numerous cellular functions that affect almost all aspects of the …

Biogenesis of nuclear pore complexes (NPCs) includes the formation of the permeability
barrier composed of phenylalanine-glycine-rich nucleoporins (FG-Nups) that regulate the …

Plasma metabolite concentrations reflect the activity of tissue metabolic pathways and their
quantitative determination may be informative about pathogenic conditions. We searched for …

Molecular chaperones are essential cellular components that aid in protein folding and
preventing the abnormal aggregation of disease-associated proteins. Mutations in one such …

Maintenance of protein homeostasis is vitally important in post-mitotic cells, particularly
neurons. Neurodegenerative diseases such as polyglutamine expansion disorders—like …

Skeletal muscle and the nervous system depend on efficient protein quality control, and they
express chaperones and cochaperones at high levels to maintain protein homeostasis …

Objective: Inherited myopathies comprise more than 200 different individually rare disease-
subtypes, but when combined together they have a high prevalence of 1 in 6,000 individuals …

Distal myopathies are genetic primary muscle disorders with a prominent weakness at onset
in hands and/or feet. The age of onset (from early childhood to adulthood), the distribution of …