Autoinflammatory diseases were first recognized nearly 20 years ago as distinct clinical and
immunological entities caused by dysregulation in the innate immune system. Since then …

The basic principle of adaptive immunity is to strictly discriminate between self and non-self,
and a central challenge to overcome is the enormous variety of pathogens that might be …

Background The study of autoinflammatory diseases has uncovered mechanisms
underlying cytokine dysregulation and inflammation. Methods We analyzed the DNA of an …

Objective Autoinflammatory type I interferonopathies, chronic atypical neutrophilic
dermatosis with lipodystrophy and elevated temperature/proteasome-associated …

The ubiquitin–proteasome system (UPS) is the primary selective degradation system in the
nuclei and cytoplasm of eukaryotic cells, required for the turnover of myriad soluble proteins …

The bowhead whale (Balaena mysticetus) is estimated to live over 200 years and is possibly
the longest-living mammal. These animals should possess protective molecular adaptations …

Autosomal recessive mutations in proteasome subunit β 8 (PSMB8), which encodes the
inducible proteasome subunit β5i, cause the immune-dysregulatory disease chronic atypical …

The ubiquitin–proteasome system (UPS) is the major intracellular and non-lysosomal
protein degradation system. Thanks to its unique capacity of eliminating old, damaged …

A critical step in preserving protein homeostasis is the recognition, binding, unfolding, and
translocation of protein substrates by six AAA-ATPase proteasome subunits (ATPase …

BACKGROUND. Monogenic IFN–mediated autoinflammatory diseases present in infancy
with systemic inflammation, an IFN response gene signature, inflammatory organ damage …