The coalescence of proteins into highly ordered aggregates is a hallmark of protein misfolding
disorders (PMDs), which, when affecting the central nervous system, lead to progressive …

Three decades after the discovery of prions as the cause of Creutzfeldt-Jakob disease and
other transmissible spongiform encephalopathies, we are still nowhere close to finding an …

Ablation of the cellular prion protein PrP C leads to a chronic demyelinating polyneuropathy
affecting Schwann cells. Neuron-restricted expression of PrP C prevents the disease 1 , …

A third of the human genome encodes N‐glycosylated proteins. These are co‐translationally
translocated into the lumen/membrane of the endoplasmic reticulum (ER) where they fold …

SRP is essential for targeting nascent chains to the endoplasmic reticulum, and it delays
nascent chain elongation in cell-free translation systems. However, the significance of this …

Mammalian cells secrete a large number of small proteins, but their mode of translocation
into the endoplasmic reticulum is not fully understood. Cotranslational translocation was …

Prion infections cause inexorable, progressive neurological dysfunction and neurodegeneration.
Expression of the cellular prion protein PrP C is required for toxicity, suggesting the …

Myelin is a multilamellar sheath generated by specialized glia called Schwann cells (SCs)
in the peripheral nervous system (PNS), which serves to protect and insulate axons for rapid …

The signal recognition particle (SRP) is a ubiquitous cytoplasmic ribonucleoprotein complex
required for the cotranslational targeting of proteins to the endoplasmic reticulum (ER). In …

Although prion infections cause cognitive impairment and neuronal death, transcriptional
and translational profiling shows progressive derangement within glia but surprisingly little …