Human wild-type and D76N β₂-microglobulin variants are significant proteotoxic and metabolic stressors for transgenic C. elegans.

ABSTRACT: β₂-microglobulin (β₂-m) is a plasma protein derived from physiological shedding of the class I major histocompatibility complex (MHCI), causing human systemic amyloidosis either due to persistently high concentrations of the wild-type (WT) protein in hemodialyzed patients, or in presence of mutations, such as D76N β₂-m, which favor protein deposition in the adulthood, despite normal plasma levels. Here we describe a new transgenic Caenorhabditis elegans (C. elegans) strain expressing human WT β₂-m at high concentrations, mimicking the condition that underlies dialysis-related amyloidosis (DRA) and we compare it to a previously established strain expressing the highly amyloidogenic D76N β₂-m at lower concentrations. Both strains

SUBMITTER: Raimondi S 

PROVIDER: S-EPMC10626158 | biostudies-literature | 2023 Nov

REPOSITORIES: biostudies-literature