[HTML][HTML] Limb-girdle muscular dystrophy (LGMD-1C) mutants of caveolin-3 undergo ubiquitination and proteasomal degradation: treatment with proteasomal inhibitors …
F Galbiati, D Volonté, C Minetti, DB Bregman… - Journal of Biological …, 2000 - ASBMB
Caveolin-3 is the principal structural protein of caveolae in striated muscle. Autosomal
dominant limb-girdle muscular dystrophy (LGMD-1C) in humans is due to mutations (ΔTFT
and Pro→ Leu) within the CAV3 gene. We have shown that LGMD-1C mutations lead to
formation of unstable aggregates of caveolin-3 that are retained intracellularly and are
rapidly degraded. The mechanism by which LGMD-1C mutants of caveolin-3 are degraded
remains unknown. Here, we show that LGMD-1C mutants of caveolin-3 undergo …
dominant limb-girdle muscular dystrophy (LGMD-1C) in humans is due to mutations (ΔTFT
and Pro→ Leu) within the CAV3 gene. We have shown that LGMD-1C mutations lead to
formation of unstable aggregates of caveolin-3 that are retained intracellularly and are
rapidly degraded. The mechanism by which LGMD-1C mutants of caveolin-3 are degraded
remains unknown. Here, we show that LGMD-1C mutants of caveolin-3 undergo …
