Items for "endoplasmic reticulum-associated degradation"

A counterintuitive approach to treat enzyme deficiencies: use of enzyme inhibitors for restoring mutant enzyme activity

Pharmacological chaperone therapy is an emerging counterintuitive approach to treat protein deficiencies resulting from mutations causing misfolded protein conformations...

Keywords: active-site-specific chaperone, endoplasmic reticulum-associated degradation, enzyme inhibitor, lysosomal storage disorders, pharmacological chaperone, protein misfolding