Ubiquitin proteasome dysfunction in human hypertrophic and dilated cardiomyopathies.

ABSTRACT:

Background

The ubiquitin proteasome system maintains a dynamic equilibrium of proteins and prevents accumulation of damaged and misfolded proteins, yet its role in human cardiac dysfunction is not well understood. The present study evaluated ubiquitin proteasome system function in human heart failure and hypertrophic cardiomyopathy (HCM).

Methods and results

Proteasome function was studied in human nonfailing donor hearts, explanted failing hearts, and myectomy samples from patients with HCM. Proteasome proteolytic activities were markedly reduced in failing and HCM hearts compared with nonfailing hearts (P<0.01). This activity was partially restored after mechanical unloading in failing hearts (P<0.01) and was significantly lower in HCM hearts with pathogenic sarcomere muta

SUBMITTER: Predmore JM 

PROVIDER: S-EPMC2857348 | biostudies-literature | 2010 Mar

REPOSITORIES: biostudies-literature